BACKGROUND Extramedullary plasmacytoma(EMP)represents one of the rarer forms of plasma cell malignancies,capable of impacting a variety of tissues and organs throughout the body.The majority of EMP cases are predomina...BACKGROUND Extramedullary plasmacytoma(EMP)represents one of the rarer forms of plasma cell malignancies,capable of impacting a variety of tissues and organs throughout the body.The majority of EMP cases are predominantly found in the head and neck region,especially within the laryngopharynx,as well as in the gastrointestinal tract.While there have been documented instances of oropharyngeal involvement in EMP cases in the academic literature,it is important to note that EMP specifically affecting the uvula is exceedingly uncommon.Furthermore,it is noteworthy that over 60% of epithelial carcinomas in the upper respiratory tract and oropharynx tend to metastasize to the cervical lymph nodes,indicating a propensity for regional spread in these types of cancers.In this context,we present a rare case of extramedullary plasmacytoma where the uvula served as the initially affected site.This case emphasizes the need for heightened awareness among clinicians regarding such unusual comorbidities,as early recognition and diagnosis can significantly influence patient management and treatment outcomes.In addition,a review of the relevant literature is included to further educate and inform healthcare professionals about this rare presentation,ultimately aiming to enhance clinical understanding and improve patient care in similar situations.CASE SUMMARY A 51-year-old man was admitted to our hospital because of a slowly enlarging neck mass.A physical examination revealed a palpable left lymph node,and magnetic resonance imaging(MRI)of the oropharynx and the neck showed a soft tissue mass in the oropharynx and enlargement of multiple lymph nodes in the neck.The soft tissue mass was diagnosed as plasmacytoma by immunohistochemical analysis.Monoclonal immunoglobulins and bone marrow biopsy showed normal results.Therefore,we diagnosed that as EMP of the uvula.After four cycles of adjuvant chemotherapy dominated by bortezomib,MRI reexamination showed a significant reduction of the mass in the oropharynx and the cervical lymph nodes.Afterwards,the λ light chain returned to normal levels.There was no evidence of evolution to multiple myeloma.CONCLUSION We have reported a rare case of extramedullary plasmacytoma with the uvula as the first affected site and the relevant literature is reviewed to improve clinicians'awareness of such rare comorbidities.展开更多
Primary gastric plasmacytoma (GP) is a rare extramedullary plasmacytoma with clinical and imaging features that are common among other gastric tumors, such as gastric adenocarcinomas, gastric stromal tumors, and lymph...Primary gastric plasmacytoma (GP) is a rare extramedullary plasmacytoma with clinical and imaging features that are common among other gastric tumors, such as gastric adenocarcinomas, gastric stromal tumors, and lymphomas. Here, we present a histologically confirmed case of primary GP examined with biphasic computed tomography (CT), magnetic resonance imaging (MRI), and endosonography. A well-circumscribed extraluminal mass appearing as homogeneous attenuation/intensity with gradual enhancement was identified on biphasic enhancement CT and MRI. This mass was hyperintense on diffusion-weighted imaging and hypointense on the apparent diffusion coefficient map, implying that water diffusion in the mass was restricted. In addition, endosonography indicated a low echogenic mass in the gastric wall. These imaging findings increase the available knowledge about imaging of this disease and provide valuable information for differentiating primary GP from common gastric tumors.展开更多
Neoplastic proliferation of plasma cells is called plasma cell dyscrasias, and these neoplasms can present as a solitary neoplasm or multiple myeloma. Extramedullary plasmacytoma, in particular pancreatic plasmacytoma...Neoplastic proliferation of plasma cells is called plasma cell dyscrasias, and these neoplasms can present as a solitary neoplasm or multiple myeloma. Extramedullary plasmacytoma, in particular pancreatic plasmacytoma, is a rare manifestation of multiple myeloma. Although computerized tomography is useful for the diagnosis of extramedullary plasmacytoma, there are no specific radiologic markers that distinguish it from adenocarcinoma. Histological confirmation by biopsy is necessary for accurate diagnosis and management of the tumor. Endosonography is the most sensitive method for the diagnosis of pancreatic tumors, and the use of fine needle aspiration by endosonography is associated with a lower risk for malignant seeding and complications. Here, we report a case of pancreatic plasmacytoma in newly identified multiple myeloma as diagnosed by endosonography. Endosonography is a reliable and rapid method for the diagnosis of extramedullary plasmacytoma. Therefore, endosonographic fine needle aspiration should be the first choice for histological evaluation when pancreatic plasmacytoma is suspected. Ideally, the pathology would be performed at the same site as endosonographic biopsy.展开更多
A 71-year-old man, with history of plasmacytoma in relapse since one year, was hospitalized for a initial presentation of acute pancreatitis and hepatitis.Although there was a heterogeneous infiltration around the pan...A 71-year-old man, with history of plasmacytoma in relapse since one year, was hospitalized for a initial presentation of acute pancreatitis and hepatitis.Although there was a heterogeneous infiltration around the pancreas head, the diagnosis of an extramedullary localization of his plasmacytoma was not made until later.This delayed diagnosis was due to the lack of specific radiologic features and the lack of dilatation of biliary ducts at the admission.A diagnosis was made with a simple ultrasound guided paracentesis of the low abundance ascites after a transjugular hepatic biopsy, an endoscopic ultrasound-guided fine needle aspiration of the pancreatic mass, and a failed attempt of biliary drainage through endoscopic retrograde cholangiopancreatography.In order to document the difficulty of this diagnosis, characteristics of 63 patients suffering from this condition and diagnosis were identified and discussed through a systematic literature search.展开更多
BACKGROUND Extramedullary plasmacytoma(EMP),a variant form of myeloma,is a rare solid plasma cell tumor that originates from the bone marrow hematopoietic tissue and accounts for about 3%of all plasma cell tumors.EMP ...BACKGROUND Extramedullary plasmacytoma(EMP),a variant form of myeloma,is a rare solid plasma cell tumor that originates from the bone marrow hematopoietic tissue and accounts for about 3%of all plasma cell tumors.EMP can affect various tissues and organs,about 90%of which is found in the head and neck.However,EMP in the reproductive organs is rare,and is difficult to be distinguished from other primary or metastatic genital tumors according to clinical symptoms and imaging findings.CASE SUMMARY Herein,we report a case with coexistence of EMP and squamous cell carcinoma in the cervix.The first histopathological report of neoplasms on the surface of the cervix and vagina showed an EMP.Both ultrasound and pelvic enhanced magnetic resonance imaging(MRI)indicated that there was a tumor in the cervix.Thus,another cervical biopsy and pathological examination were performed,which indicated EMP combined with squamous cell carcinoma.Then,the patient underwent extensive total hysterectomy(type C1)+systemic lymph node dissection and received 25 external pelvic irradiations with a dose of 50 Gy following surgery.During 2-year follow-up,no recurrence was reported.CONCLUSION In conclusion,EMP involving the reproductive system is relatively rare.In this case,MRI,B-ultrasound,and cervical canal scraping were used to further determine the diagnosis of EMP combined with squamous cell carcinoma.The patient had improved prognosis after appropriate treatments.展开更多
BACKGROUND: Extramedullary pancreatic plasmacytoma treated with bortezomib is rarely reported. METHODS: We admitted a 53-year-old woman with an asymptomatic mass above the left clavicle for over three months, then an ...BACKGROUND: Extramedullary pancreatic plasmacytoma treated with bortezomib is rarely reported. METHODS: We admitted a 53-year-old woman with an asymptomatic mass above the left clavicle for over three months, then an asymptomatic swelling of the pancreas was found. A biopsy on the mass and a fine needle aspiration of the pancreas were performed. The diagnosis of extramedullary plasmacytoma (EMP) was made. The patient was initially treated with combination chemotherapy consisting of vincristine, doxorubicin and dexamethasone (VAD regimen). She progressed to painless jaundice during the chemotherapy. Then she was treated with bortezomib and hyper-dose dexamethasone. As a result, she had a near complete remission. RESULTS: The data demonstrated that the diagnosis was EMP of the pancreas. The patient responded very well to bortezomib, while failing to respond to the traditional chemotherapy regimen of VAD. CONCLUSION: EMP of the pancreas is rare. This case gives evidence for an excellent response of EMP of the pancreas to bortezomib. (Hepatobiliary Pancreat Dis Int 2009; 8: 329-331)展开更多
BACKGROUND Non-secretory multiple myeloma(MM)is a rare condition that accounts for only 3%of MM cases and is defined by normal serum and urine immunofixation and a normal serum free light chain ratio.Non-secretory MM ...BACKGROUND Non-secretory multiple myeloma(MM)is a rare condition that accounts for only 3%of MM cases and is defined by normal serum and urine immunofixation and a normal serum free light chain ratio.Non-secretory MM with multiple extramedullary plasmacytomas derived from endobronchial lesions is extremely rare and can be misdiagnosed as metastasis of solid cancer.CASE SUMMARY A 36-year-old man presented with progressive facial swelling and nasal congestion with cough.Various imaging studies revealed an endobronchial mass in the left bronchus and a large left maxillary mass with multiple destructive bone metastatic lesions.He initially presented with lung cancer and multiple metastases.However,pathologic reports showed multiple extramedullary plasmacytomas in the left maxilla and the left bronchus.There was no change in the serum and urine monoclonal protein levels,and no abnormalities were observed in laboratory examinations,including hemoglobin,calcium,and creatinine levels.The bone marrow was hypercellular,with 13.49%plasma cells.The patient was diagnosed with non-secretory MM expressed as multiple extramedullary plasmacytomas with endobronchial lesions in a rare location.Radiation therapy for symptomatic lesions with high-dose dexamethasone was started,and the size of the left maxillary sinus lesion dramatically decreased.In the future,chemotherapy will be administered to control lesions in other areas.CONCLUSION We present a rare case of non-secretory MM with multiple extramedullary plasmacytoma with an endobronchial lesion.展开更多
Objective： To investigate potential prognostic factors affecting patient outcomes and to evaluate the optimal methods and effects of radiotherapy（RT） in the management of extramedullary plasmacytoma（EMP）.Methods...Objective： To investigate potential prognostic factors affecting patient outcomes and to evaluate the optimal methods and effects of radiotherapy（RT） in the management of extramedullary plasmacytoma（EMP）.Methods： Data from 55 patients with EMP between November 1999 and August 2015 were collected. The median age was 51（range, 22–77） years. The median tumor size was 3.5（range, 1.0–15.0） cm. The median applied dose was 50.0（range, 30.0–70.0） Gy. Thirty-nine patients（70.9%） presented with disease in the head or neck region. Twelve patients received RT alone, 9 received surgery（S） alone, 3 received chemotherapy（CT） alone, and 3 patients did not receive any treatment. Combination therapies were applied in 28 patients.Results：The median follow-up duration was 56 months.The 5-year local recurrence-free survival（LRFS）,multiple myeloma-free survival（MMFS）,progression-free survival（PFS）and overall survival（OS）rates were 79.8%,78.6%,65.2% and 76.0%,respectively.Univariate analysis revealed that RT was a favourable factor for all examined endpoints.Furthermore,head and neck EMPs were associated with superior LRFS,MMFS and PFS.Tumor size〈4 cm was associated with superior MMFS,PFS and OS;serum M protein negativity was associated with superior MMFS and PFS;age≥50 years and local recurrence were associated with poor MMFS.The dose≥45 Gy group exhibited superior 5-year LRFS,MMFS and PFS rates（94.7%,94.4%,90.0%,respectively）,while the corresponding values for the dose〈45 Gy group were 62.5%（P=0.008）,53.3%（P=0.036）and 41.7%（P〈0.001）.Conclusions：Involved-site RT of at least 45 Gy should be considered for EMP.Furthermore,patients with head and neck EMP,tumor size〈4 cm,age〈50 years and serum M protein negativity had better outcomes.展开更多
BACKGROUND Extramedullary plasmacytoma(EMP)is a rare kind of soft tissue plasma cell neoplasm without bone marrow involvement;this type of plasma cell neoplasm involves a lack of other systemic characteristics of mult...BACKGROUND Extramedullary plasmacytoma(EMP)is a rare kind of soft tissue plasma cell neoplasm without bone marrow involvement;this type of plasma cell neoplasm involves a lack of other systemic characteristics of multiple myeloma.Primary pulmonary plasmacytoma(PPP),with no specific clinical manifestations,is an exceedingly rare type of EMP.Because of its complexity,PPP is often difficult to diagnose,and there is no report in the literature on cases accompanied by overlap syndrome(OS).CASE SUMMARY A 61-year-old woman without a familial lung cancer history was admitted to our hospital in 2018,for intermittent cough,expectoration,and a stuffy feeling in the chest for 50 years;these symptoms appeared intermittently,especially occurred after being cold,and had been aggravated for the last 10 d.She was diagnosed with pulmonary fibrosis and emphysema,bronchiectasis,OS,and autoimmune hepatic cirrhosis in 2017.A pulmonary examination revealed rough breath sounds in both lungs;other physical examinations found no obvious abnormalities.A routine laboratory work-up showed decreased haemoglobin,increased ESR,and abnormal GGT,ALT,Ig G,γ-globulin,κ-light chain,λ-light chain,rheumatoid factor,and autoimmune antibodies.Emission computed tomography demonstrated abnormally concentrated 99 m Tc-MDP.Chest computed tomography revealed a soft tissue mass in the middle and lower lobes of the right lung.After right middle and inferior lobe resection with complete mediastinal lymph node dissection,immunohistochemical analysis revealed an isolated pulmonary plasmacytoma.The patient received chemotherapy for more than 1.5 years and remains in good general condition.CONCLUSION PPP is a type of EMP,and we report an exceedingly rare presentation of PPP accompanied by OS.展开更多
AIM: To assess the feasibility of the combination of helical tomotherapy(HT) and a concurrent systemic targeted therapy in patients with solitary plasmacytoma (SP) with the aim to decrease toxicity while improving the...AIM: To assess the feasibility of the combination of helical tomotherapy(HT) and a concurrent systemic targeted therapy in patients with solitary plasmacytoma (SP) with the aim to decrease toxicity while improving therapeutic efficacy. METHODS: Six patients with biologically, histologically, and radiologically confirmed SP were treated using HT and a systemic targeted treatment concomitantly. Total dose was 40 Gy/20 fractions. Four patients received 4 cycles of concurrent lenalidomide-dexamethasone combination and two patients were treated with concomitant bortezomib-dexamethasone. All toxicities were described using the Common Terminology Criteria for Adverse Effects v3.0.RESULTS: Five patients had a bone tumor and one patient had an isolated pancreatic mass. Five patients presented with pain, one had neurologic symptoms related to medullary compression, which was treated by an emergency surgery. Median age was 59.5 years (range, 50-74 years). All patients had initial positron emission tomography-computed tomographys, three patients had total body bone magnetic resonance imaging examination, and three patients had computed tomodensitometry scans. The toxicity profile was excellent with no higher than grade 1 toxicity. Four of the six patients experienced a partial radiological response, four had complete response on positions emission tomography and 5/6 patients experienced a complete relief of their symptoms 4 mo after treatment. At a median follow-up of 18 mo, 5/6 patients were controlled clinically, radiologically, and biologically. CONCLUSION: Using HT, we could deliver a highly conformal irradiation concurrently with a molecularly targeted therapy. This association yielded in a high response rate and a low toxicity. A prospective study with longer follow-up will help determining the true benefit of such strategy.展开更多
Among plasma cell disorders, solitary plasmacytoma (solitany-plasmacytoma of bone, SPB and extramedullary plasmacytoma, EMP) is rare as compared with mulitiple myeloma (MM). Furthermore.the relationship between solita...Among plasma cell disorders, solitary plasmacytoma (solitany-plasmacytoma of bone, SPB and extramedullary plasmacytoma, EMP) is rare as compared with mulitiple myeloma (MM). Furthermore.the relationship between solitary plasmacytoma and MM remains unclear.Between 1960 and 1994, 24 patients with SPB and 20 with EMP were treated. The criteria for diagonosis were: (1) No evidence of other lesions based on clinical and radiologic examinations;(2) Biopsy evidence of a plasma cell neoplasm; (3) Bone marrow biopsy specimen with negative findings (less than 10% plasma cell); (4) No anemia, hypercalcemia or renal involvement. The average follow-up period was 112 months (from 6 to 360 months). Fifty-four percent of patients with SPB and 40% of patients with EMP developed MM, however, there was no significant statistical difference between SPB and EMP (P <0.05).We suggested that solitary plasmacytomas be classified as two types, latent and aggressive. The former was histologically well-differentiated plasmacytomas. The latter was poorly differentiated tumors which easily progress to MM. The treatment of choice is wide excision or thorough curettage, by cryogenic necrosis with liquid nitrogen or cautery of the bony wall with phenol and the cavity filled with bone grafts or cement. All patients with apparently isolated plasmacytoma should he given if the tumor turns out to be poorly differentiated, in order to delay their progression to MM.展开更多
Extramedullary plasmacytoma(EPM) is a plasma cell tumor arising outside of the bone marrow.Solitary EMP is an uncommon neoplasm and rarely occurs in the retroperitoneum and lacks distinctive clinical manifestations.We...Extramedullary plasmacytoma(EPM) is a plasma cell tumor arising outside of the bone marrow.Solitary EMP is an uncommon neoplasm and rarely occurs in the retroperitoneum and lacks distinctive clinical manifestations.We report a 26-year-old man with a solitary EMP in the retroperitoneum and discuss its clinical features,diagnosis and treatment.展开更多
BACKGROUND Solitary plasmacytoma in the left rib is rare and can cause chest discomfort such as chest pain and tightness,and its clinical manifestations are not typical,so it is often misdiagnosed.We report a case of ...BACKGROUND Solitary plasmacytoma in the left rib is rare and can cause chest discomfort such as chest pain and tightness,and its clinical manifestations are not typical,so it is often misdiagnosed.We report a case of left costal plasmacytoma misdiagnosed as angina pectoris.We also review the literature and provide suggestions as to how to avoid misdiagnosis.CASE SUMMARY A 77-year-old man with a history of intermittent chest tightness for 3 years presented with pain in the left chest for 1 wk and was admitted to hospital.The cardiologists initially diagnosed angina pectoris but the findings of coronary angiography were not consistent with the symptoms.Computed tomography showed that the left eighth rib mass was accompanied by bone destruction.The patient was transferred to our department for further treatment.Preoperative biopsy indicated that the lesion was possibly malignant,and elective surgery was performed to remove the lesion.The size of the tumor was about 4 cm.The tumor was spindle-shaped and protruded into the pleural cavity,without invading the lungs.Postoperative pathology confirmed that the left rib lesion was plasmacytoma.After 14 mo follow-up,the patient died of systemic metastasis.CONCLUSION Left rib solitary plasmacytoma is a rare disease confined to a specific rib and can cause local pain.Attention should be paid to the differential diagnosis of angina pectoris to avoid misdiagnosis.展开更多
BACKGROUND Solitary bone plasmacytoma(SBP)of the upper cervical spine is a rare diagnosis.The exact role of surgery for SBP remains unclear.CASE SUMMARY We present the first case of SBP of the C2.A 69-year-old Chinese...BACKGROUND Solitary bone plasmacytoma(SBP)of the upper cervical spine is a rare diagnosis.The exact role of surgery for SBP remains unclear.CASE SUMMARY We present the first case of SBP of the C2.A 69-year-old Chinese woman presented with severe neck pain and limitation of rotative activity for 2 mo.She underwent anterior one-stage debridement combined with cement augmentation in the C2 to reconstruct stability of the spine.The patient did not receive postoperative radiotherapy.She now remains disease free with no neck pain or neurological deficit after follow-up of 3 years.CONCLUSION Anterior one-stage debridement combined with cement augmentation of the upper cervical spine may be an alternative treatment for SBP.展开更多
BACKGROUND Solitary plasmacytoma and unicentric Castleman disease(UCD)are rare lymphoproliferative disorders characterized by monoclonal plasma cells and a single set of locally enlarged lymph nodes,respectively.CASE ...BACKGROUND Solitary plasmacytoma and unicentric Castleman disease(UCD)are rare lymphoproliferative disorders characterized by monoclonal plasma cells and a single set of locally enlarged lymph nodes,respectively.CASE SUMMARY A 48-year-old Han Chinese man presented to our department with a neck mass and progressive foreign body sensation in his throat.18F-FDG positron emission tomography revealed focally increased radioactivity centered around the hyoid,and computed tomography(CT)revealed osteolytic lesions.Histopathology revealed Castleman-like features and CD138/CD38-positive mature plasma cells.Systemic work-up ruled out the possibility of POEMS syndrome,lymphoma,and multiple myeloma,leading to a final diagnosis of solitary hyoid plasmacytoma with UCD.The patient underwent partial hyoid resection and selective neck dissection,followed by intensity-modulated radiotherapy.99mTc-MDP singlephoton emission computed tomography/CT reevaluation showed neither local recurrence nor distant bone metastasis at the 40-mo follow-up.CONCLUSION The diagnostic process and differential diagnosis of this rare case provided valuable educational information to clinicians.展开更多
Extramedullary plasmacytoma （EMP） is rare in multiple myeloma, especially kidneys are involved. We report one case, including diagnosis and treatment. The purpose of this paper is to provide an attention on the extr...Extramedullary plasmacytoma （EMP） is rare in multiple myeloma, especially kidneys are involved. We report one case, including diagnosis and treatment. The purpose of this paper is to provide an attention on the extramedullary plasmacytoma disease, reducing the misdiagnose with this disease.展开更多
Pancreatic plasmacytoma is a rare entity & present with features of mass lesion of pancreas.We presented an interesting case of pancreatic plasmacytoma with severe abdominal pain and cholestatic symptoms.This case...Pancreatic plasmacytoma is a rare entity & present with features of mass lesion of pancreas.We presented an interesting case of pancreatic plasmacytoma with severe abdominal pain and cholestatic symptoms.This case highlights the importance of considering pancreatic plasmacytoma in differential diagnosis of patients with cholestatic jaundice & abdominal pain and that not all pancreatic head mass are adenocarcinoma,so biopsy is advisable.展开更多
BACKGROUND Extramedullary plasmacytoma(EMP)of the gastrointestinal tract is an extremely rare disease.Clinical manifestations of EMPs are varied and depend on the location and progression of the tumor.CASE SUMMARY Her...BACKGROUND Extramedullary plasmacytoma(EMP)of the gastrointestinal tract is an extremely rare disease.Clinical manifestations of EMPs are varied and depend on the location and progression of the tumor.CASE SUMMARY Here,we firstly report a case of intestinal perforation with abdominal abscess caused by EMP of the small intestine in a 55-year-old female patient.The patient received emergency surgery immediately after the necessary preoperative procedures.During the operation,EMP was found to have caused the perforation of the small intestine and the formation of multiple abscesses in the abdominal cavity.Partial resection of the small intestine with peritoneal irrigation and drainage was performed.EMP was finally confirmed by postoperative histopathology and laboratory tests.Additionally,we performed a literature review of gastrointestinal EMP to obtain a deeper understanding of this disease.CONCLUSION EMP of the small intestine may have spontaneous perforation,which requires emergency surgery.Surgical resection can obtain good therapeutic effects.展开更多
BACKGROUND Therapy-related acute promyelocytic leukemia(t-APL)is a rare complication observed in solitary bone plasmacytoma(SBP),and SBP after radiotherapy evolving to APL harboring the FMS-like tyrosine kinase 3-inte...BACKGROUND Therapy-related acute promyelocytic leukemia(t-APL)is a rare complication observed in solitary bone plasmacytoma(SBP),and SBP after radiotherapy evolving to APL harboring the FMS-like tyrosine kinase 3-internal tandem duplication(FLT3-ITD)mutation has never been reported.Here,we present the first case reported until now.CASE SUMMARY We describe a 64-year-old woman who presented with lumbar pain and was initially diagnosed with SBP.However,after one year of radiotherapy treatment,this patient experienced a long-standing bone-marrow-suppressive period and finally developed APL harboring the FLT3-ITD mutation,as confirmed by analyses of clinical features,bone marrow morphology,flow cytometry,cytogenetic examination,and molecular biology.On admission,the patient had disseminated intravascular coagulation and intracranial hemorrhage,and the peripheral blood and bone marrow smear displayed abundant abnormal promyelocytes.Unfortunately,she died when the definite diagnosis was made.CONCLUSION The patient with t-APL harboring FLT3-ITD mutation evolving from SBP after radiotherapy had not been reported and had poor clinical outcomes.FLT3-ITD mutation in t-APL may be a potential pathogenesis of leukemogenesis.We should consider the potential risk of secondary neoplasms in SBP patients after radiotherapy.展开更多
OBJECTIVE To evaluate the MR imaging features of solitary plasmacytomas of the spine. METHODS The MR images of 7 patients with histologically proven solitary plasmacytomas of the spine were reviewed. RESULTS All tumor...OBJECTIVE To evaluate the MR imaging features of solitary plasmacytomas of the spine. METHODS The MR images of 7 patients with histologically proven solitary plasmacytomas of the spine were reviewed. RESULTS All tumors showed predominantly isoto hypointensity relative to muscle on Tl-weighted MR images and intermediate signal intensity between muscle and fat on T2- weighted images. Curvilinear low signal intensity structures were seen within the lesions on T1 and T2-weighted images in five tumors. Moderate to strong enhancement was seen in all 6 tumors that underwent contrast enhanced MR examination. All tumors showed areas of high signal intensity on T2-weighted images and heterogeneous enhancement, except the 2 largest tumors without pathologic fracture in the sacrum. Intervertebral discs were preserved in all tumors. CONCLUSION The MR imaging features that suggest plasmacytorna of the spine include predominant intermediate signal intensity on T2-weighted images, curvilinear low signal intensity structures, moderate to strong enhancement, relatively homogeneous appearance if there are no pathologic fracture and preservation of the intervertebral discs.展开更多
基金Supported by Hunan Innovative Province Construction Special Task Book,No.2021SK4050Innovation Platform and Talent Program of Hunan Province,No.2023JJ30609.
文摘BACKGROUND Extramedullary plasmacytoma(EMP)represents one of the rarer forms of plasma cell malignancies,capable of impacting a variety of tissues and organs throughout the body.The majority of EMP cases are predominantly found in the head and neck region,especially within the laryngopharynx,as well as in the gastrointestinal tract.While there have been documented instances of oropharyngeal involvement in EMP cases in the academic literature,it is important to note that EMP specifically affecting the uvula is exceedingly uncommon.Furthermore,it is noteworthy that over 60% of epithelial carcinomas in the upper respiratory tract and oropharynx tend to metastasize to the cervical lymph nodes,indicating a propensity for regional spread in these types of cancers.In this context,we present a rare case of extramedullary plasmacytoma where the uvula served as the initially affected site.This case emphasizes the need for heightened awareness among clinicians regarding such unusual comorbidities,as early recognition and diagnosis can significantly influence patient management and treatment outcomes.In addition,a review of the relevant literature is included to further educate and inform healthcare professionals about this rare presentation,ultimately aiming to enhance clinical understanding and improve patient care in similar situations.CASE SUMMARY A 51-year-old man was admitted to our hospital because of a slowly enlarging neck mass.A physical examination revealed a palpable left lymph node,and magnetic resonance imaging(MRI)of the oropharynx and the neck showed a soft tissue mass in the oropharynx and enlargement of multiple lymph nodes in the neck.The soft tissue mass was diagnosed as plasmacytoma by immunohistochemical analysis.Monoclonal immunoglobulins and bone marrow biopsy showed normal results.Therefore,we diagnosed that as EMP of the uvula.After four cycles of adjuvant chemotherapy dominated by bortezomib,MRI reexamination showed a significant reduction of the mass in the oropharynx and the cervical lymph nodes.Afterwards,the λ light chain returned to normal levels.There was no evidence of evolution to multiple myeloma.CONCLUSION We have reported a rare case of extramedullary plasmacytoma with the uvula as the first affected site and the relevant literature is reviewed to improve clinicians'awareness of such rare comorbidities.
基金Supported by The Research Projects of Public Technology Application of Science and Technology of Shaoxing City,No.2013B70080
文摘Primary gastric plasmacytoma (GP) is a rare extramedullary plasmacytoma with clinical and imaging features that are common among other gastric tumors, such as gastric adenocarcinomas, gastric stromal tumors, and lymphomas. Here, we present a histologically confirmed case of primary GP examined with biphasic computed tomography (CT), magnetic resonance imaging (MRI), and endosonography. A well-circumscribed extraluminal mass appearing as homogeneous attenuation/intensity with gradual enhancement was identified on biphasic enhancement CT and MRI. This mass was hyperintense on diffusion-weighted imaging and hypointense on the apparent diffusion coefficient map, implying that water diffusion in the mass was restricted. In addition, endosonography indicated a low echogenic mass in the gastric wall. These imaging findings increase the available knowledge about imaging of this disease and provide valuable information for differentiating primary GP from common gastric tumors.
文摘Neoplastic proliferation of plasma cells is called plasma cell dyscrasias, and these neoplasms can present as a solitary neoplasm or multiple myeloma. Extramedullary plasmacytoma, in particular pancreatic plasmacytoma, is a rare manifestation of multiple myeloma. Although computerized tomography is useful for the diagnosis of extramedullary plasmacytoma, there are no specific radiologic markers that distinguish it from adenocarcinoma. Histological confirmation by biopsy is necessary for accurate diagnosis and management of the tumor. Endosonography is the most sensitive method for the diagnosis of pancreatic tumors, and the use of fine needle aspiration by endosonography is associated with a lower risk for malignant seeding and complications. Here, we report a case of pancreatic plasmacytoma in newly identified multiple myeloma as diagnosed by endosonography. Endosonography is a reliable and rapid method for the diagnosis of extramedullary plasmacytoma. Therefore, endosonographic fine needle aspiration should be the first choice for histological evaluation when pancreatic plasmacytoma is suspected. Ideally, the pathology would be performed at the same site as endosonographic biopsy.
文摘A 71-year-old man, with history of plasmacytoma in relapse since one year, was hospitalized for a initial presentation of acute pancreatitis and hepatitis.Although there was a heterogeneous infiltration around the pancreas head, the diagnosis of an extramedullary localization of his plasmacytoma was not made until later.This delayed diagnosis was due to the lack of specific radiologic features and the lack of dilatation of biliary ducts at the admission.A diagnosis was made with a simple ultrasound guided paracentesis of the low abundance ascites after a transjugular hepatic biopsy, an endoscopic ultrasound-guided fine needle aspiration of the pancreatic mass, and a failed attempt of biliary drainage through endoscopic retrograde cholangiopancreatography.In order to document the difficulty of this diagnosis, characteristics of 63 patients suffering from this condition and diagnosis were identified and discussed through a systematic literature search.
基金Science and Technology Project of Zunyi City and The First People's Hospital of Zunyi,No.187,2018.
文摘BACKGROUND Extramedullary plasmacytoma(EMP),a variant form of myeloma,is a rare solid plasma cell tumor that originates from the bone marrow hematopoietic tissue and accounts for about 3%of all plasma cell tumors.EMP can affect various tissues and organs,about 90%of which is found in the head and neck.However,EMP in the reproductive organs is rare,and is difficult to be distinguished from other primary or metastatic genital tumors according to clinical symptoms and imaging findings.CASE SUMMARY Herein,we report a case with coexistence of EMP and squamous cell carcinoma in the cervix.The first histopathological report of neoplasms on the surface of the cervix and vagina showed an EMP.Both ultrasound and pelvic enhanced magnetic resonance imaging(MRI)indicated that there was a tumor in the cervix.Thus,another cervical biopsy and pathological examination were performed,which indicated EMP combined with squamous cell carcinoma.Then,the patient underwent extensive total hysterectomy(type C1)+systemic lymph node dissection and received 25 external pelvic irradiations with a dose of 50 Gy following surgery.During 2-year follow-up,no recurrence was reported.CONCLUSION In conclusion,EMP involving the reproductive system is relatively rare.In this case,MRI,B-ultrasound,and cervical canal scraping were used to further determine the diagnosis of EMP combined with squamous cell carcinoma.The patient had improved prognosis after appropriate treatments.
文摘BACKGROUND: Extramedullary pancreatic plasmacytoma treated with bortezomib is rarely reported. METHODS: We admitted a 53-year-old woman with an asymptomatic mass above the left clavicle for over three months, then an asymptomatic swelling of the pancreas was found. A biopsy on the mass and a fine needle aspiration of the pancreas were performed. The diagnosis of extramedullary plasmacytoma (EMP) was made. The patient was initially treated with combination chemotherapy consisting of vincristine, doxorubicin and dexamethasone (VAD regimen). She progressed to painless jaundice during the chemotherapy. Then she was treated with bortezomib and hyper-dose dexamethasone. As a result, she had a near complete remission. RESULTS: The data demonstrated that the diagnosis was EMP of the pancreas. The patient responded very well to bortezomib, while failing to respond to the traditional chemotherapy regimen of VAD. CONCLUSION: EMP of the pancreas is rare. This case gives evidence for an excellent response of EMP of the pancreas to bortezomib. (Hepatobiliary Pancreat Dis Int 2009; 8: 329-331)
基金Supported by the Clinical Medicine Research Institute at Chosun University Hospital (2019)
文摘BACKGROUND Non-secretory multiple myeloma(MM)is a rare condition that accounts for only 3%of MM cases and is defined by normal serum and urine immunofixation and a normal serum free light chain ratio.Non-secretory MM with multiple extramedullary plasmacytomas derived from endobronchial lesions is extremely rare and can be misdiagnosed as metastasis of solid cancer.CASE SUMMARY A 36-year-old man presented with progressive facial swelling and nasal congestion with cough.Various imaging studies revealed an endobronchial mass in the left bronchus and a large left maxillary mass with multiple destructive bone metastatic lesions.He initially presented with lung cancer and multiple metastases.However,pathologic reports showed multiple extramedullary plasmacytomas in the left maxilla and the left bronchus.There was no change in the serum and urine monoclonal protein levels,and no abnormalities were observed in laboratory examinations,including hemoglobin,calcium,and creatinine levels.The bone marrow was hypercellular,with 13.49%plasma cells.The patient was diagnosed with non-secretory MM expressed as multiple extramedullary plasmacytomas with endobronchial lesions in a rare location.Radiation therapy for symptomatic lesions with high-dose dexamethasone was started,and the size of the left maxillary sinus lesion dramatically decreased.In the future,chemotherapy will be administered to control lesions in other areas.CONCLUSION We present a rare case of non-secretory MM with multiple extramedullary plasmacytoma with an endobronchial lesion.
文摘Objective： To investigate potential prognostic factors affecting patient outcomes and to evaluate the optimal methods and effects of radiotherapy（RT） in the management of extramedullary plasmacytoma（EMP）.Methods： Data from 55 patients with EMP between November 1999 and August 2015 were collected. The median age was 51（range, 22–77） years. The median tumor size was 3.5（range, 1.0–15.0） cm. The median applied dose was 50.0（range, 30.0–70.0） Gy. Thirty-nine patients（70.9%） presented with disease in the head or neck region. Twelve patients received RT alone, 9 received surgery（S） alone, 3 received chemotherapy（CT） alone, and 3 patients did not receive any treatment. Combination therapies were applied in 28 patients.Results：The median follow-up duration was 56 months.The 5-year local recurrence-free survival（LRFS）,multiple myeloma-free survival（MMFS）,progression-free survival（PFS）and overall survival（OS）rates were 79.8%,78.6%,65.2% and 76.0%,respectively.Univariate analysis revealed that RT was a favourable factor for all examined endpoints.Furthermore,head and neck EMPs were associated with superior LRFS,MMFS and PFS.Tumor size〈4 cm was associated with superior MMFS,PFS and OS;serum M protein negativity was associated with superior MMFS and PFS;age≥50 years and local recurrence were associated with poor MMFS.The dose≥45 Gy group exhibited superior 5-year LRFS,MMFS and PFS rates（94.7%,94.4%,90.0%,respectively）,while the corresponding values for the dose〈45 Gy group were 62.5%（P=0.008）,53.3%（P=0.036）and 41.7%（P〈0.001）.Conclusions：Involved-site RT of at least 45 Gy should be considered for EMP.Furthermore,patients with head and neck EMP,tumor size〈4 cm,age〈50 years and serum M protein negativity had better outcomes.
基金the National Nature Science Foundation of China,No.81500430 and No.U1304802the Science and Technology Planning Project of Henan Province,No.192102310045,No.182102310544,No.182102310566,and No.182102310573the Henan Medical Science and Technology Tackling Project,No.2018020337,No.2018020332,No.2018020334,and No.2018020320。
文摘BACKGROUND Extramedullary plasmacytoma(EMP)is a rare kind of soft tissue plasma cell neoplasm without bone marrow involvement;this type of plasma cell neoplasm involves a lack of other systemic characteristics of multiple myeloma.Primary pulmonary plasmacytoma(PPP),with no specific clinical manifestations,is an exceedingly rare type of EMP.Because of its complexity,PPP is often difficult to diagnose,and there is no report in the literature on cases accompanied by overlap syndrome(OS).CASE SUMMARY A 61-year-old woman without a familial lung cancer history was admitted to our hospital in 2018,for intermittent cough,expectoration,and a stuffy feeling in the chest for 50 years;these symptoms appeared intermittently,especially occurred after being cold,and had been aggravated for the last 10 d.She was diagnosed with pulmonary fibrosis and emphysema,bronchiectasis,OS,and autoimmune hepatic cirrhosis in 2017.A pulmonary examination revealed rough breath sounds in both lungs;other physical examinations found no obvious abnormalities.A routine laboratory work-up showed decreased haemoglobin,increased ESR,and abnormal GGT,ALT,Ig G,γ-globulin,κ-light chain,λ-light chain,rheumatoid factor,and autoimmune antibodies.Emission computed tomography demonstrated abnormally concentrated 99 m Tc-MDP.Chest computed tomography revealed a soft tissue mass in the middle and lower lobes of the right lung.After right middle and inferior lobe resection with complete mediastinal lymph node dissection,immunohistochemical analysis revealed an isolated pulmonary plasmacytoma.The patient received chemotherapy for more than 1.5 years and remains in good general condition.CONCLUSION PPP is a type of EMP,and we report an exceedingly rare presentation of PPP accompanied by OS.
文摘AIM: To assess the feasibility of the combination of helical tomotherapy(HT) and a concurrent systemic targeted therapy in patients with solitary plasmacytoma (SP) with the aim to decrease toxicity while improving therapeutic efficacy. METHODS: Six patients with biologically, histologically, and radiologically confirmed SP were treated using HT and a systemic targeted treatment concomitantly. Total dose was 40 Gy/20 fractions. Four patients received 4 cycles of concurrent lenalidomide-dexamethasone combination and two patients were treated with concomitant bortezomib-dexamethasone. All toxicities were described using the Common Terminology Criteria for Adverse Effects v3.0.RESULTS: Five patients had a bone tumor and one patient had an isolated pancreatic mass. Five patients presented with pain, one had neurologic symptoms related to medullary compression, which was treated by an emergency surgery. Median age was 59.5 years (range, 50-74 years). All patients had initial positron emission tomography-computed tomographys, three patients had total body bone magnetic resonance imaging examination, and three patients had computed tomodensitometry scans. The toxicity profile was excellent with no higher than grade 1 toxicity. Four of the six patients experienced a partial radiological response, four had complete response on positions emission tomography and 5/6 patients experienced a complete relief of their symptoms 4 mo after treatment. At a median follow-up of 18 mo, 5/6 patients were controlled clinically, radiologically, and biologically. CONCLUSION: Using HT, we could deliver a highly conformal irradiation concurrently with a molecularly targeted therapy. This association yielded in a high response rate and a low toxicity. A prospective study with longer follow-up will help determining the true benefit of such strategy.
文摘Among plasma cell disorders, solitary plasmacytoma (solitany-plasmacytoma of bone, SPB and extramedullary plasmacytoma, EMP) is rare as compared with mulitiple myeloma (MM). Furthermore.the relationship between solitary plasmacytoma and MM remains unclear.Between 1960 and 1994, 24 patients with SPB and 20 with EMP were treated. The criteria for diagonosis were: (1) No evidence of other lesions based on clinical and radiologic examinations;(2) Biopsy evidence of a plasma cell neoplasm; (3) Bone marrow biopsy specimen with negative findings (less than 10% plasma cell); (4) No anemia, hypercalcemia or renal involvement. The average follow-up period was 112 months (from 6 to 360 months). Fifty-four percent of patients with SPB and 40% of patients with EMP developed MM, however, there was no significant statistical difference between SPB and EMP (P <0.05).We suggested that solitary plasmacytomas be classified as two types, latent and aggressive. The former was histologically well-differentiated plasmacytomas. The latter was poorly differentiated tumors which easily progress to MM. The treatment of choice is wide excision or thorough curettage, by cryogenic necrosis with liquid nitrogen or cautery of the bony wall with phenol and the cavity filled with bone grafts or cement. All patients with apparently isolated plasmacytoma should he given if the tumor turns out to be poorly differentiated, in order to delay their progression to MM.
文摘Extramedullary plasmacytoma(EPM) is a plasma cell tumor arising outside of the bone marrow.Solitary EMP is an uncommon neoplasm and rarely occurs in the retroperitoneum and lacks distinctive clinical manifestations.We report a 26-year-old man with a solitary EMP in the retroperitoneum and discuss its clinical features,diagnosis and treatment.
文摘BACKGROUND Solitary plasmacytoma in the left rib is rare and can cause chest discomfort such as chest pain and tightness,and its clinical manifestations are not typical,so it is often misdiagnosed.We report a case of left costal plasmacytoma misdiagnosed as angina pectoris.We also review the literature and provide suggestions as to how to avoid misdiagnosis.CASE SUMMARY A 77-year-old man with a history of intermittent chest tightness for 3 years presented with pain in the left chest for 1 wk and was admitted to hospital.The cardiologists initially diagnosed angina pectoris but the findings of coronary angiography were not consistent with the symptoms.Computed tomography showed that the left eighth rib mass was accompanied by bone destruction.The patient was transferred to our department for further treatment.Preoperative biopsy indicated that the lesion was possibly malignant,and elective surgery was performed to remove the lesion.The size of the tumor was about 4 cm.The tumor was spindle-shaped and protruded into the pleural cavity,without invading the lungs.Postoperative pathology confirmed that the left rib lesion was plasmacytoma.After 14 mo follow-up,the patient died of systemic metastasis.CONCLUSION Left rib solitary plasmacytoma is a rare disease confined to a specific rib and can cause local pain.Attention should be paid to the differential diagnosis of angina pectoris to avoid misdiagnosis.
文摘BACKGROUND Solitary bone plasmacytoma(SBP)of the upper cervical spine is a rare diagnosis.The exact role of surgery for SBP remains unclear.CASE SUMMARY We present the first case of SBP of the C2.A 69-year-old Chinese woman presented with severe neck pain and limitation of rotative activity for 2 mo.She underwent anterior one-stage debridement combined with cement augmentation in the C2 to reconstruct stability of the spine.The patient did not receive postoperative radiotherapy.She now remains disease free with no neck pain or neurological deficit after follow-up of 3 years.CONCLUSION Anterior one-stage debridement combined with cement augmentation of the upper cervical spine may be an alternative treatment for SBP.
基金Supported by Key Program of the Medical Engineering Cross Research Fund of Shanghai Jiao Tong University,No.YG2019QNA55Tou-Yan Innovation Team Program of Heilongjiang Province,No.2019-15。
文摘BACKGROUND Solitary plasmacytoma and unicentric Castleman disease(UCD)are rare lymphoproliferative disorders characterized by monoclonal plasma cells and a single set of locally enlarged lymph nodes,respectively.CASE SUMMARY A 48-year-old Han Chinese man presented to our department with a neck mass and progressive foreign body sensation in his throat.18F-FDG positron emission tomography revealed focally increased radioactivity centered around the hyoid,and computed tomography(CT)revealed osteolytic lesions.Histopathology revealed Castleman-like features and CD138/CD38-positive mature plasma cells.Systemic work-up ruled out the possibility of POEMS syndrome,lymphoma,and multiple myeloma,leading to a final diagnosis of solitary hyoid plasmacytoma with UCD.The patient underwent partial hyoid resection and selective neck dissection,followed by intensity-modulated radiotherapy.99mTc-MDP singlephoton emission computed tomography/CT reevaluation showed neither local recurrence nor distant bone metastasis at the 40-mo follow-up.CONCLUSION The diagnostic process and differential diagnosis of this rare case provided valuable educational information to clinicians.
文摘Extramedullary plasmacytoma （EMP） is rare in multiple myeloma, especially kidneys are involved. We report one case, including diagnosis and treatment. The purpose of this paper is to provide an attention on the extramedullary plasmacytoma disease, reducing the misdiagnose with this disease.
文摘Pancreatic plasmacytoma is a rare entity & present with features of mass lesion of pancreas.We presented an interesting case of pancreatic plasmacytoma with severe abdominal pain and cholestatic symptoms.This case highlights the importance of considering pancreatic plasmacytoma in differential diagnosis of patients with cholestatic jaundice & abdominal pain and that not all pancreatic head mass are adenocarcinoma,so biopsy is advisable.
基金Supported by the Natural Science Foundation of Education Department of Liaoning Province,No.QNZR2020008.
文摘BACKGROUND Extramedullary plasmacytoma(EMP)of the gastrointestinal tract is an extremely rare disease.Clinical manifestations of EMPs are varied and depend on the location and progression of the tumor.CASE SUMMARY Here,we firstly report a case of intestinal perforation with abdominal abscess caused by EMP of the small intestine in a 55-year-old female patient.The patient received emergency surgery immediately after the necessary preoperative procedures.During the operation,EMP was found to have caused the perforation of the small intestine and the formation of multiple abscesses in the abdominal cavity.Partial resection of the small intestine with peritoneal irrigation and drainage was performed.EMP was finally confirmed by postoperative histopathology and laboratory tests.Additionally,we performed a literature review of gastrointestinal EMP to obtain a deeper understanding of this disease.CONCLUSION EMP of the small intestine may have spontaneous perforation,which requires emergency surgery.Surgical resection can obtain good therapeutic effects.
基金Natural Science Foundation of Zhejiang Province,No.LY19H290003Zhejiang Provincial Medical and Health Science and Technology Project,No.2020KY196and Foundation of Zhejiang Province Chinese Medicine Science and Technology Planes,No.2017ZB030.
文摘BACKGROUND Therapy-related acute promyelocytic leukemia(t-APL)is a rare complication observed in solitary bone plasmacytoma(SBP),and SBP after radiotherapy evolving to APL harboring the FMS-like tyrosine kinase 3-internal tandem duplication(FLT3-ITD)mutation has never been reported.Here,we present the first case reported until now.CASE SUMMARY We describe a 64-year-old woman who presented with lumbar pain and was initially diagnosed with SBP.However,after one year of radiotherapy treatment,this patient experienced a long-standing bone-marrow-suppressive period and finally developed APL harboring the FLT3-ITD mutation,as confirmed by analyses of clinical features,bone marrow morphology,flow cytometry,cytogenetic examination,and molecular biology.On admission,the patient had disseminated intravascular coagulation and intracranial hemorrhage,and the peripheral blood and bone marrow smear displayed abundant abnormal promyelocytes.Unfortunately,she died when the definite diagnosis was made.CONCLUSION The patient with t-APL harboring FLT3-ITD mutation evolving from SBP after radiotherapy had not been reported and had poor clinical outcomes.FLT3-ITD mutation in t-APL may be a potential pathogenesis of leukemogenesis.We should consider the potential risk of secondary neoplasms in SBP patients after radiotherapy.
文摘OBJECTIVE To evaluate the MR imaging features of solitary plasmacytomas of the spine. METHODS The MR images of 7 patients with histologically proven solitary plasmacytomas of the spine were reviewed. RESULTS All tumors showed predominantly isoto hypointensity relative to muscle on Tl-weighted MR images and intermediate signal intensity between muscle and fat on T2- weighted images. Curvilinear low signal intensity structures were seen within the lesions on T1 and T2-weighted images in five tumors. Moderate to strong enhancement was seen in all 6 tumors that underwent contrast enhanced MR examination. All tumors showed areas of high signal intensity on T2-weighted images and heterogeneous enhancement, except the 2 largest tumors without pathologic fracture in the sacrum. Intervertebral discs were preserved in all tumors. CONCLUSION The MR imaging features that suggest plasmacytorna of the spine include predominant intermediate signal intensity on T2-weighted images, curvilinear low signal intensity structures, moderate to strong enhancement, relatively homogeneous appearance if there are no pathologic fracture and preservation of the intervertebral discs.
