BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a rare malignant mesenchymal tumor with a poor prognosis.It mainly occurs in the extremities,trunk,head and neck,and retroperitoneum regions.Owing to the lack of ...BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a rare malignant mesenchymal tumor with a poor prognosis.It mainly occurs in the extremities,trunk,head and neck,and retroperitoneum regions.Owing to the lack of specific clinical manifestations and imaging features,UPS diagnosis mainly depends on pathological and immunohistochemical examinations for exclusive diagnosis.Here we report an extremely rare case of high-grade UPS in the common bile duct(CBD).There are limited available data on such cases.CASE SUMMARY A 70-year-old woman was admitted to our department with yellow eyes and urine accompanied by upper abdominal distending pain for 2 wk.Her laboratory data suggested significantly elevated hepatorenal function levels.The imaging data revealed calculous cholecystitis,intrahepatic and extrahepatic bile duct dilation with extrahepatic bile duct calculi,and a space-occupying lesion at the distal CBD.After endoscopic biliary stenting and symptomatic support therapy,CBD exploration and biopsy were performed.The frozen section indicated malignant spindle cell tumor of the CBD mass,and further radical pancreaticoduodenectomy was performed.Finally,the neoplasm was diagnosed as a high-grade UPS combined with the light-microscopic morphology and immunohistochemical results.CONCLUSION This extremely rare case highlighted the need for increasing physicians'vigilance,reducing the odds of misdiagnosis,and providing appropriate treatment strategies.展开更多
Undifferentiated pleomorphic sarcoma is an extremely rare malignant thyroid tumor.Thyroid sarcoma differs from common malignant thyroid tumors,such as thyroid follicular cell carcinoma.It is usually highly malignant,p...Undifferentiated pleomorphic sarcoma is an extremely rare malignant thyroid tumor.Thyroid sarcoma differs from common malignant thyroid tumors,such as thyroid follicular cell carcinoma.It is usually highly malignant,progresses rapidly,and is prone to remote metastasis.Currently,there is no standard protocol for the treatment of thyroid sarcomas,and most treatment effects are unsatisfactory.Argon-helium cryoablation is an important method of local treatment that is widely used in patients with unresectable advanced tumors.However,owing to the low incidence of thyroid sarcomas,there are no relevant literature reports on the treatment of thyroid sarcomas using cryoablation in China.This study reports the case of a patient with undifferentiated pleomorphic sarcoma of the thyroid gland who was treated with argon-helium cryoablation,and the immediate outcome was good.Based on a review of relevant literature,we discussed the effectiveness and safety of argon-helium cryoablation treatment,to provide clinical guidance and references for the treatment of patients with thyroid sarcoma.展开更多
BACKGROUND:A malignant fibrous histiocytoma,recently referred to as an undifferentiated pleomorphic sarcoma (UPS),is very rare in the liver,and only 34 cases of primary hepatic malignant fibrous histiocytoma have been...BACKGROUND:A malignant fibrous histiocytoma,recently referred to as an undifferentiated pleomorphic sarcoma (UPS),is very rare in the liver,and only 34 cases of primary hepatic malignant fibrous histiocytoma have been reported in the English literature. METHODS:We report a rare case of a hepatic UPS presenting as a unilocular cystic lesion with respect to histopathologic features,the newly revised diagnostic criteria,and the differential diagnosis. RESULTS:A 60-year-old man presented for evaluation of epigastric pain of 7 months duration.Abdominal computed tomography revealed a hypodense,unilocular cystic mass in the right lobe of the liver,measuring 14.0×11.1 cm.A right lobectomy was performed.Grossly,the cut surface showed a single,well-circumscribed unilocular cystic tumor mass containing dark red-brown necrotic debris and blood clots, which occupied most of the mass.Microscopically,the tumor consisted of haphazardly arranged mononuclear pleomorphic tumor cells,admixed with abundant osteoclast- like multinucleated giant cells.Immunohistochemically,the tumor cells expressed vimentin only.The histopathologic and immunohistochemical findings were compatible with a UPS.The patient is alive and well 41 months after surgery without recurrence. CONCLUSIONS:Clinically,most of the hepatic UPSs are solid masses.Only two cases have presented as multilocular cystic masses.A primary hepatic UPS presenting as a unilocular cyst has never been reported.A UPS should be included in the differential diagnosis of unilocular cystic lesions in the liver.展开更多
We present a case of spontaneous undifferentiated/unclassified sarcoma,of a pleomorphic subtype formerly known as malignant fibrous histiocytoma(UPS/MFH),arising from the pancreas of a laboratory rat.The mass was exci...We present a case of spontaneous undifferentiated/unclassified sarcoma,of a pleomorphic subtype formerly known as malignant fibrous histiocytoma(UPS/MFH),arising from the pancreas of a laboratory rat.The mass was excised after laparotomy from a 6‐month‐old female laboratory Wistar rat.It presented a giant multilobulated mass of irregular shape,which had arisen from the pancreas and occupied almost the entire peritoneal cavity.Histologically the tumor was characterized by a highly variable morphological pattern,with frequent transitions from storiform to pleomorphic areas.An extensive immunohistochemical examination revealed no specific lines of differentiation.Immunohistochemical positivity was observed only to MIB‐1(high Ki‐67 proliferation index),vimentin and CD68 antibodies.The diagnosis was compatible with UPS/MFH.To the best of our knowledge,the present case is the first report of a spontaneous primary UPS/MFH arising from the pancreas of a laboratory rat.展开更多
BACKGROUND Papillary thyroid cancer(PTC)is the most common malignant tumor of the thyroid.However,the coexistence of PTC and sarcoma in one patient is rare.In this article,we report the case of a patient who presented...BACKGROUND Papillary thyroid cancer(PTC)is the most common malignant tumor of the thyroid.However,the coexistence of PTC and sarcoma in one patient is rare.In this article,we report the case of a patient who presented with both PTC and undifferentiated pleomorphic sarcoma(UPS),which has not been previously reported in the online Medline database(PubMed).CASE SUMMARY A 71-year-old man was admitted to our hospital for a mass on the right side of his neck for one month,which rapidly enlarged within 2 wk with distending pain.The patient was diagnosed with a thyroid malignancy by fine-needle aspiration and underwent total thyroidectomy and bilateral central lymph node dissection.Histology and immunohistochemistry revealed features of both PTC and UPS.The thyroid cancer 8 gene detection kit results showed BRAF and telomerase reverse transcriptase mutations.The disease progressed rapidly,and the patient died four months after surgery from extensive lung metastasis.CONCLUSION Our report highlights the patient’s pathological characteristics and related genetic mutations.Due to the rapid development and poor prognosis of cooccurring PTC and sarcoma,it is important for clinical physicians and pathologists to raise awareness of this type of tumor.展开更多
BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a type of soft tissue sarcoma,the histologic origin and differentiation direction of which are still unclear.There are few treatment options for UPS other than su...BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a type of soft tissue sarcoma,the histologic origin and differentiation direction of which are still unclear.There are few treatment options for UPS other than surgery.Herein we describe a patient who had multiple recurrences of UPS postoperatively,but R0 resection was achieved by local hyperthermia combined with chemotherapy,thus providing a new treatment approach for similar situations.CASE SUMMARY A 65-year-old man sought evaluation from a physician for a mass on his right back.After surgery,the pathologic diagnosis was fibrosarcoma.During the follow-up evaluations until 2021,the patient had four relapses of varying degrees.Postoperative pathology confirmed the recurrence of UPS on the right back.In March 2021,he underwent local hyperthermia combined with two cycles of chemotherapy for recurring lesions.After magnetic resonance imaging reexamination and preoperative examination,the patient chose surgery again.During the operation,the tumors were easy to excise,the amount of bleeding decreased significantly,and the pathologic evaluation confirmed that one of the specimens was an R0 excision.CONCLUSION Local hyperthermia combined with chemotherapy enables R0 resection to be achieved in patients with advanced UPS recurrence.展开更多
Primary undifferentiated pleomorphic sarcoma (UPS) of the small intestine is extremely rare. The prognosis of UPS is basically poor, and particular, when accompanied with metastatic lesions. This paper reports the cas...Primary undifferentiated pleomorphic sarcoma (UPS) of the small intestine is extremely rare. The prognosis of UPS is basically poor, and particular, when accompanied with metastatic lesions. This paper reports the case of a long-term survivor of primary UPS of the jejunum with lymph node metastases and a skip lesion in the jejunum. The patient was a 50-year-old Japanese man who presented with a chief complaint of breathlessness. Small bowel X-ray series revealed an approximately 4-cm size with protruded lesion (image shows a filling defect) in the proximal jejunum. Based on a presumptive diagnosis of the hemorrhagic small bowel tumor, he underwent a laparotomy. A tumor was observed in the jejunum at approximately 90-cm from the Treitz ligament;some swollen mesenteric lymph nodules were also observed. Segmental resection of the jejunum was performed 20-140 cm from the Treitz ligament. A complete surgical excision with en-bloc regional lymph node dissection was performed. The final histopathological diagnosis was UPS of the jejunum with metastatic lymph nodes and a skip lesion in the jejunum. The postoperative course was uneventful. The patient was not given adjuvant chemotherapy and was discharged on postoperative day 16. He is currently well without any evidence of recurrence for >10 years after the operation.展开更多
Undifferentiated pleomorphic sarcoma (UPS), also called malignant fibrous histiocytoma (MFH), belongs to the soft tissue tumors and is more likely in elder patients. UPS currently accounts for approximately 18% of cut...Undifferentiated pleomorphic sarcoma (UPS), also called malignant fibrous histiocytoma (MFH), belongs to the soft tissue tumors and is more likely in elder patients. UPS currently accounts for approximately 18% of cutaneous soft tissue sarcoma except Kaposi sarcoma. The most common sites are the extremities and head and neck. UPS is rapidly enlarging, and easily invades subcutaneous tissue and muscle without overlying skin changes. UPS tumors, which are confined to subcutaneous tissue, have been reported to be less than 10%. On the other hand, the involvement of the epidermis, occasionally with ulceration, is rare. Here, we describe a case of UPS of the cheek with surface ulceration. An 83-year-old man presented with a 1-year history of a red nodule on his left cheek. The size of the nodule gradually increased. Clinical examination revealed a 55 × 45 mm red, hard mass in the center of his left cheek with a large cratershaped ulcer involving the subcutaneous tissue. The bottom of the ulcer contained necrotic tissue. No cervical lymph nodes were palpable. A biopsy revealed a high-grade, pleomorphic spindle cell neoplasm and frequent abnormal mitotic figures within the dermis. We temporarily diagnosed and suspected a soft tissue sarcoma, but we could not rule out spindle cell squamous cell carcinoma (SCSCC). The patient underwent tumor resection and skin grafting. The tumor had reached the muscle layer of his left cheek with invasion and ulceration of the overlying skin. Histopathological examination of the totally resected tumor revealed a neoplasm of mesenchymal origin, rich in cells with morphology and immunohistochemical characteristics compatible with UPS. Malignant cells were not found in the epidermis. Immunohistochemical analyses were positive for vimentin, α-anti-trypsin, α-smooth muscle actin (α-SMA), and CD10, and negative for cytokeratins (CAM5.2 and AE1/AE3), lysozyme, epithelial membrane antigen (EMA), CD34, S-100, desmin, and caldesmon. Two months after total resection, the tumor recurred, and the patient was treated with radiation therapy. However, he died 2 months later from multiple lung metastases. We thought that the metastases were from the UPS, because his prostatic carcinoma had not progressed. According to published literatures, the UPS rarely accompanies with surface ulceration. On the other hand, SCC commonly occurs on sun-exposed sites of the head and neck with surface changes including scaling, ulceration, crusting, and the presence of a cutaneous horn. In our case, it was very difficult to distinguish UPS from SCSCC by clinical examination and conventional histopathological staining (e.g. hematoxylin and eosin). Therefore, the immunohistochemistry was required to obtain the final diagnosis.展开更多
Soft tissue sarcomas are unusual malignancies comprising 1% of cancer diagnoses in the United States. Undifferentiated pleomorphic sarcoma accounts for approximately 5% of sarcomas occurring in adults. The most common...Soft tissue sarcomas are unusual malignancies comprising 1% of cancer diagnoses in the United States. Undifferentiated pleomorphic sarcoma accounts for approximately 5% of sarcomas occurring in adults. The most common site of metastasis is the lung, with other sites being bone, the brain, and the liver. Metastasis to the gastrointestinal tract has rarely been documented. We present an unusual case of high-grade pleomorphic sarcoma with metastasis to the stomach, complicated by upper gastrointestinal bleeding.展开更多
基金Supported by the People’s Livelihood Science and Technology Innovation Project of the Bureau of Science and Technology of Jiaxing City,No.2021AD30091.
文摘BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a rare malignant mesenchymal tumor with a poor prognosis.It mainly occurs in the extremities,trunk,head and neck,and retroperitoneum regions.Owing to the lack of specific clinical manifestations and imaging features,UPS diagnosis mainly depends on pathological and immunohistochemical examinations for exclusive diagnosis.Here we report an extremely rare case of high-grade UPS in the common bile duct(CBD).There are limited available data on such cases.CASE SUMMARY A 70-year-old woman was admitted to our department with yellow eyes and urine accompanied by upper abdominal distending pain for 2 wk.Her laboratory data suggested significantly elevated hepatorenal function levels.The imaging data revealed calculous cholecystitis,intrahepatic and extrahepatic bile duct dilation with extrahepatic bile duct calculi,and a space-occupying lesion at the distal CBD.After endoscopic biliary stenting and symptomatic support therapy,CBD exploration and biopsy were performed.The frozen section indicated malignant spindle cell tumor of the CBD mass,and further radical pancreaticoduodenectomy was performed.Finally,the neoplasm was diagnosed as a high-grade UPS combined with the light-microscopic morphology and immunohistochemical results.CONCLUSION This extremely rare case highlighted the need for increasing physicians'vigilance,reducing the odds of misdiagnosis,and providing appropriate treatment strategies.
文摘Undifferentiated pleomorphic sarcoma is an extremely rare malignant thyroid tumor.Thyroid sarcoma differs from common malignant thyroid tumors,such as thyroid follicular cell carcinoma.It is usually highly malignant,progresses rapidly,and is prone to remote metastasis.Currently,there is no standard protocol for the treatment of thyroid sarcomas,and most treatment effects are unsatisfactory.Argon-helium cryoablation is an important method of local treatment that is widely used in patients with unresectable advanced tumors.However,owing to the low incidence of thyroid sarcomas,there are no relevant literature reports on the treatment of thyroid sarcomas using cryoablation in China.This study reports the case of a patient with undifferentiated pleomorphic sarcoma of the thyroid gland who was treated with argon-helium cryoablation,and the immediate outcome was good.Based on a review of relevant literature,we discussed the effectiveness and safety of argon-helium cryoablation treatment,to provide clinical guidance and references for the treatment of patients with thyroid sarcoma.
文摘BACKGROUND:A malignant fibrous histiocytoma,recently referred to as an undifferentiated pleomorphic sarcoma (UPS),is very rare in the liver,and only 34 cases of primary hepatic malignant fibrous histiocytoma have been reported in the English literature. METHODS:We report a rare case of a hepatic UPS presenting as a unilocular cystic lesion with respect to histopathologic features,the newly revised diagnostic criteria,and the differential diagnosis. RESULTS:A 60-year-old man presented for evaluation of epigastric pain of 7 months duration.Abdominal computed tomography revealed a hypodense,unilocular cystic mass in the right lobe of the liver,measuring 14.0×11.1 cm.A right lobectomy was performed.Grossly,the cut surface showed a single,well-circumscribed unilocular cystic tumor mass containing dark red-brown necrotic debris and blood clots, which occupied most of the mass.Microscopically,the tumor consisted of haphazardly arranged mononuclear pleomorphic tumor cells,admixed with abundant osteoclast- like multinucleated giant cells.Immunohistochemically,the tumor cells expressed vimentin only.The histopathologic and immunohistochemical findings were compatible with a UPS.The patient is alive and well 41 months after surgery without recurrence. CONCLUSIONS:Clinically,most of the hepatic UPSs are solid masses.Only two cases have presented as multilocular cystic masses.A primary hepatic UPS presenting as a unilocular cyst has never been reported.A UPS should be included in the differential diagnosis of unilocular cystic lesions in the liver.
文摘We present a case of spontaneous undifferentiated/unclassified sarcoma,of a pleomorphic subtype formerly known as malignant fibrous histiocytoma(UPS/MFH),arising from the pancreas of a laboratory rat.The mass was excised after laparotomy from a 6‐month‐old female laboratory Wistar rat.It presented a giant multilobulated mass of irregular shape,which had arisen from the pancreas and occupied almost the entire peritoneal cavity.Histologically the tumor was characterized by a highly variable morphological pattern,with frequent transitions from storiform to pleomorphic areas.An extensive immunohistochemical examination revealed no specific lines of differentiation.Immunohistochemical positivity was observed only to MIB‐1(high Ki‐67 proliferation index),vimentin and CD68 antibodies.The diagnosis was compatible with UPS/MFH.To the best of our knowledge,the present case is the first report of a spontaneous primary UPS/MFH arising from the pancreas of a laboratory rat.
文摘BACKGROUND Papillary thyroid cancer(PTC)is the most common malignant tumor of the thyroid.However,the coexistence of PTC and sarcoma in one patient is rare.In this article,we report the case of a patient who presented with both PTC and undifferentiated pleomorphic sarcoma(UPS),which has not been previously reported in the online Medline database(PubMed).CASE SUMMARY A 71-year-old man was admitted to our hospital for a mass on the right side of his neck for one month,which rapidly enlarged within 2 wk with distending pain.The patient was diagnosed with a thyroid malignancy by fine-needle aspiration and underwent total thyroidectomy and bilateral central lymph node dissection.Histology and immunohistochemistry revealed features of both PTC and UPS.The thyroid cancer 8 gene detection kit results showed BRAF and telomerase reverse transcriptase mutations.The disease progressed rapidly,and the patient died four months after surgery from extensive lung metastasis.CONCLUSION Our report highlights the patient’s pathological characteristics and related genetic mutations.Due to the rapid development and poor prognosis of cooccurring PTC and sarcoma,it is important for clinical physicians and pathologists to raise awareness of this type of tumor.
文摘BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a type of soft tissue sarcoma,the histologic origin and differentiation direction of which are still unclear.There are few treatment options for UPS other than surgery.Herein we describe a patient who had multiple recurrences of UPS postoperatively,but R0 resection was achieved by local hyperthermia combined with chemotherapy,thus providing a new treatment approach for similar situations.CASE SUMMARY A 65-year-old man sought evaluation from a physician for a mass on his right back.After surgery,the pathologic diagnosis was fibrosarcoma.During the follow-up evaluations until 2021,the patient had four relapses of varying degrees.Postoperative pathology confirmed the recurrence of UPS on the right back.In March 2021,he underwent local hyperthermia combined with two cycles of chemotherapy for recurring lesions.After magnetic resonance imaging reexamination and preoperative examination,the patient chose surgery again.During the operation,the tumors were easy to excise,the amount of bleeding decreased significantly,and the pathologic evaluation confirmed that one of the specimens was an R0 excision.CONCLUSION Local hyperthermia combined with chemotherapy enables R0 resection to be achieved in patients with advanced UPS recurrence.
文摘Primary undifferentiated pleomorphic sarcoma (UPS) of the small intestine is extremely rare. The prognosis of UPS is basically poor, and particular, when accompanied with metastatic lesions. This paper reports the case of a long-term survivor of primary UPS of the jejunum with lymph node metastases and a skip lesion in the jejunum. The patient was a 50-year-old Japanese man who presented with a chief complaint of breathlessness. Small bowel X-ray series revealed an approximately 4-cm size with protruded lesion (image shows a filling defect) in the proximal jejunum. Based on a presumptive diagnosis of the hemorrhagic small bowel tumor, he underwent a laparotomy. A tumor was observed in the jejunum at approximately 90-cm from the Treitz ligament;some swollen mesenteric lymph nodules were also observed. Segmental resection of the jejunum was performed 20-140 cm from the Treitz ligament. A complete surgical excision with en-bloc regional lymph node dissection was performed. The final histopathological diagnosis was UPS of the jejunum with metastatic lymph nodes and a skip lesion in the jejunum. The postoperative course was uneventful. The patient was not given adjuvant chemotherapy and was discharged on postoperative day 16. He is currently well without any evidence of recurrence for >10 years after the operation.
文摘Undifferentiated pleomorphic sarcoma (UPS), also called malignant fibrous histiocytoma (MFH), belongs to the soft tissue tumors and is more likely in elder patients. UPS currently accounts for approximately 18% of cutaneous soft tissue sarcoma except Kaposi sarcoma. The most common sites are the extremities and head and neck. UPS is rapidly enlarging, and easily invades subcutaneous tissue and muscle without overlying skin changes. UPS tumors, which are confined to subcutaneous tissue, have been reported to be less than 10%. On the other hand, the involvement of the epidermis, occasionally with ulceration, is rare. Here, we describe a case of UPS of the cheek with surface ulceration. An 83-year-old man presented with a 1-year history of a red nodule on his left cheek. The size of the nodule gradually increased. Clinical examination revealed a 55 × 45 mm red, hard mass in the center of his left cheek with a large cratershaped ulcer involving the subcutaneous tissue. The bottom of the ulcer contained necrotic tissue. No cervical lymph nodes were palpable. A biopsy revealed a high-grade, pleomorphic spindle cell neoplasm and frequent abnormal mitotic figures within the dermis. We temporarily diagnosed and suspected a soft tissue sarcoma, but we could not rule out spindle cell squamous cell carcinoma (SCSCC). The patient underwent tumor resection and skin grafting. The tumor had reached the muscle layer of his left cheek with invasion and ulceration of the overlying skin. Histopathological examination of the totally resected tumor revealed a neoplasm of mesenchymal origin, rich in cells with morphology and immunohistochemical characteristics compatible with UPS. Malignant cells were not found in the epidermis. Immunohistochemical analyses were positive for vimentin, α-anti-trypsin, α-smooth muscle actin (α-SMA), and CD10, and negative for cytokeratins (CAM5.2 and AE1/AE3), lysozyme, epithelial membrane antigen (EMA), CD34, S-100, desmin, and caldesmon. Two months after total resection, the tumor recurred, and the patient was treated with radiation therapy. However, he died 2 months later from multiple lung metastases. We thought that the metastases were from the UPS, because his prostatic carcinoma had not progressed. According to published literatures, the UPS rarely accompanies with surface ulceration. On the other hand, SCC commonly occurs on sun-exposed sites of the head and neck with surface changes including scaling, ulceration, crusting, and the presence of a cutaneous horn. In our case, it was very difficult to distinguish UPS from SCSCC by clinical examination and conventional histopathological staining (e.g. hematoxylin and eosin). Therefore, the immunohistochemistry was required to obtain the final diagnosis.
文摘Soft tissue sarcomas are unusual malignancies comprising 1% of cancer diagnoses in the United States. Undifferentiated pleomorphic sarcoma accounts for approximately 5% of sarcomas occurring in adults. The most common site of metastasis is the lung, with other sites being bone, the brain, and the liver. Metastasis to the gastrointestinal tract has rarely been documented. We present an unusual case of high-grade pleomorphic sarcoma with metastasis to the stomach, complicated by upper gastrointestinal bleeding.