Atypical fibroxanthomas (AFX) are rare skin tumors. These generally are superficial tumors, usually <3 cm red, fleshy, ulcerated skin lesions, that characteristically occur on sun-damaged skin, sometimes in immunoc...Atypical fibroxanthomas (AFX) are rare skin tumors. These generally are superficial tumors, usually <3 cm red, fleshy, ulcerated skin lesions, that characteristically occur on sun-damaged skin, sometimes in immunocompromised or previously irradiated patients. These are part of a spectrum of more aggressive fibro-histiocytic neoplasms. In the older literature, these have been termed aggressive or metastatic AFX, but currently these have been reclassified as pleomorphic dermal sarcomas (PDS) and systemic undifferentiated pleomorphic sarcoma (UPS, formerly malignant fibrohistiocytic sarcoma, MFH). We present the case of a 64-year old woman who developed a deeply invasive PDS on the vertex of her scalp invading to the galea, with in-transit scalp metastases. Very little information is available about optimal treatment of metastatic PDS lesions. The patient was initially treated with 2 cycles of epirubicin/ifosfamide chemotherapy, resulting in life-threatening complications. A pretreatment peripheral blood sample was sent for CTC-derived colony assay. This sample grew 8 colonies from 10 ml blood. The tumor failed to respond to epirubicin and ifosfamide, and after several months of hospitalization, a second peripheral blood CTC-derived colony assay grew >376 colonies. The patient could not tolerate additional chemotherapy. She was therefore treated with the oral targeted agent pazopanib. The patient developed a dramatic biopsy-confirmed complete response. After 11 months of pazopanib treatment, a repeat CTC-derived culture sample grew only 8 colonies/10 ml blood. The complete response to pazopanib is still ongoing at over 41 months. To our knowledge, this is the first demonstration of clinical complete response of a PDS tumor following targeted therapy. An additional novel feature was the demonstration that CTC-derived colonies could be grown from the blood of a PDS patient. The number of colonies appeared to correlate with the clinical treatment response and seemed to function as a potential prognostic marker.展开更多
Introduction: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are one spectrum of rare cutaneous neoplasms that typically arise in sun-exposed skin of older population. AFX/PDS is essentially diagnos...Introduction: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are one spectrum of rare cutaneous neoplasms that typically arise in sun-exposed skin of older population. AFX/PDS is essentially diagnosis of exclusion requiring Immunohistochemical work-up to exclude other types of tumors. Case Report: We present a case involving an ulcerated solitary lesion on the scalp of an elderly man. Histological examination revealed that the dermal tumor was composed of large pleomorphic, epithelioid, and spindle cells with clear cytoplasm. These cells were negative for cytokeratins, melanocytes and smooth muscle markers, but positive for CD10. These findings are consistent with a diagnosis of clear cell (CC) PDS. Conclusion: PDS is a low-grade malignancy that can recur locally and metastasize, which is distinguished from AFX by its larger size and the presence of aggressive histopathologic features including deeper invasion into the subcutaneous tissue, tumor necrosis, and lymphovascular and/or perineural involvement. Among several histopathologic variants, the CC variant is extremely rare with only two cases of PDS reported in the literature to date.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
BACKGROUND Pleomorphic leiomyosarcomas make up around 8.6%of all leiomyosarcomas.They behave aggressively and often have poor prognoses.They can affect the gastrointestinal tract and retroperitoneum.To date,pleomorphi...BACKGROUND Pleomorphic leiomyosarcomas make up around 8.6%of all leiomyosarcomas.They behave aggressively and often have poor prognoses.They can affect the gastrointestinal tract and retroperitoneum.To date,pleomorphic leiomyosarcoma involving the mesocolon have been reported in nine patients.CASE SUMMARY The patient was a 44-year-old man with a history of pleomorphic leiomyosarcoma of the left maxilla with metastasis to the lung and liver.His most recent positron emission tomography-computed tomography(PET-CT)scan showed uptake in the ascending and transverse colons.A colonoscopy revealed a 5.0 cm×3.5 cm×3.0 cm pedunculated polyp in the ascending colon.The polyp was removed using hot snare polypectomy technique and retrieved with Rothnet.Histopathologic examination of the polyp showed a metastatic pleomorphic leiomyosarcoma.CONCLUSION Uptake(s)on PET-CT in a patient with pleomorphic leiomyosarcoma should raise suspicion for metastasis.展开更多
Objective： Lung carcinoma with spindle and （or） giant cell （LCSG） is a rare epithelial malignant tumor. The aim of our study is to investigate the clinicopathological and prognostic characteristics of 17 cases of...Objective： Lung carcinoma with spindle and （or） giant cell （LCSG） is a rare epithelial malignant tumor. The aim of our study is to investigate the clinicopathological and prognostic characteristics of 17 cases of LCSGs. Methods： Among 421 patients underwent resection of lung carcinomas, 17 cases of LCSG were studied for clinical, gross and histological parameters. Follow-up information was obtained and analyzed to clarify prognostically significant parameters. Results： The LCSG patients consisted of 15 males and 2 females, with the age ranging from 45 to 78 years （median, 58 years）; 5 cases of stage Ⅰ, 3 of stage Ⅱ, 9 of stage Ⅲ by pathological TNM staging; 2 cases of exclusively spindle cell carcinoma, 5 cases of lung carcinoma with spindle cell, 10 cases of lung carcinoma with giant-cell carcinoma. Cough, chest distress, or chest pain were the most common presenting symptoms, occurring in 15 patients （88.2%）. Of 5 patients in stage Ⅰ, 4 were alive and free of relapse for more than 5 years. The difference in survival was statistically significant between LCSG and squamous cell carcinoma patients （median survival, 36 vs. 61 months; P = 0.027）. Lymph node metastasis and carcinoma with giant cell were the hazardous factors impacting postoperative prognosis of LCSG patients. Conclusion： LCSG patients in early stage may have an optimistic outcome. Lung carcinomas with giant cell displayed multiple cell components in histopathology, and poor outcome due to more lymph node involved.展开更多
背景与目的:含梭形/巨细胞成分肺癌(carcinoma with spindle/giantcells,LCSG)是一种少见的恶性上皮肿瘤,相关文献报道较少。本研究目的是探讨LCSG临床病理特点及预后。方法:总结、分析17例手术治疗的LCSG患者的临床和组织病理学资料,...背景与目的:含梭形/巨细胞成分肺癌(carcinoma with spindle/giantcells,LCSG)是一种少见的恶性上皮肿瘤,相关文献报道较少。本研究目的是探讨LCSG临床病理特点及预后。方法:总结、分析17例手术治疗的LCSG患者的临床和组织病理学资料,并与同期手术的肺癌患者进行比较。利用生存曲线进行生存分析,Cox风险比例模型分析影响预后的独立因素。结果:LCSG患者男女比例为15∶2,年龄45～78岁,中位年龄58岁;pTNM分期Ⅰ期5例,Ⅱ期3例,Ⅲ期9例;病理类型梭形细胞癌2例,含梭形细胞癌(均伴一种恶性上皮成分,鳞癌3例、腺癌和大细胞癌各1例)5例,含巨细胞癌(分别有5例伴一种上皮癌、5例伴两种上皮癌)10例;4例Ⅰ期患者术后无瘤生存5年以上;LCSG患者比同期鳞癌的预后差(中位生存36个月和61个月,P=0.027),含巨细胞肺癌患者的预后比含梭形细胞肺癌(包括梭形细胞癌)患者差(中位生存18个月和64个月,P=0.026)。多因素分析显示淋巴结分期和含巨细胞癌是影响LCSG患者预后的危险因素。结论:LCSG作为一种独特类型的肺癌,其中含巨细胞的肺癌成分复杂,预后较差。展开更多
文摘Atypical fibroxanthomas (AFX) are rare skin tumors. These generally are superficial tumors, usually <3 cm red, fleshy, ulcerated skin lesions, that characteristically occur on sun-damaged skin, sometimes in immunocompromised or previously irradiated patients. These are part of a spectrum of more aggressive fibro-histiocytic neoplasms. In the older literature, these have been termed aggressive or metastatic AFX, but currently these have been reclassified as pleomorphic dermal sarcomas (PDS) and systemic undifferentiated pleomorphic sarcoma (UPS, formerly malignant fibrohistiocytic sarcoma, MFH). We present the case of a 64-year old woman who developed a deeply invasive PDS on the vertex of her scalp invading to the galea, with in-transit scalp metastases. Very little information is available about optimal treatment of metastatic PDS lesions. The patient was initially treated with 2 cycles of epirubicin/ifosfamide chemotherapy, resulting in life-threatening complications. A pretreatment peripheral blood sample was sent for CTC-derived colony assay. This sample grew 8 colonies from 10 ml blood. The tumor failed to respond to epirubicin and ifosfamide, and after several months of hospitalization, a second peripheral blood CTC-derived colony assay grew >376 colonies. The patient could not tolerate additional chemotherapy. She was therefore treated with the oral targeted agent pazopanib. The patient developed a dramatic biopsy-confirmed complete response. After 11 months of pazopanib treatment, a repeat CTC-derived culture sample grew only 8 colonies/10 ml blood. The complete response to pazopanib is still ongoing at over 41 months. To our knowledge, this is the first demonstration of clinical complete response of a PDS tumor following targeted therapy. An additional novel feature was the demonstration that CTC-derived colonies could be grown from the blood of a PDS patient. The number of colonies appeared to correlate with the clinical treatment response and seemed to function as a potential prognostic marker.
文摘Introduction: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are one spectrum of rare cutaneous neoplasms that typically arise in sun-exposed skin of older population. AFX/PDS is essentially diagnosis of exclusion requiring Immunohistochemical work-up to exclude other types of tumors. Case Report: We present a case involving an ulcerated solitary lesion on the scalp of an elderly man. Histological examination revealed that the dermal tumor was composed of large pleomorphic, epithelioid, and spindle cells with clear cytoplasm. These cells were negative for cytokeratins, melanocytes and smooth muscle markers, but positive for CD10. These findings are consistent with a diagnosis of clear cell (CC) PDS. Conclusion: PDS is a low-grade malignancy that can recur locally and metastasize, which is distinguished from AFX by its larger size and the presence of aggressive histopathologic features including deeper invasion into the subcutaneous tissue, tumor necrosis, and lymphovascular and/or perineural involvement. Among several histopathologic variants, the CC variant is extremely rare with only two cases of PDS reported in the literature to date.
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BACKGROUND Pleomorphic leiomyosarcomas make up around 8.6%of all leiomyosarcomas.They behave aggressively and often have poor prognoses.They can affect the gastrointestinal tract and retroperitoneum.To date,pleomorphic leiomyosarcoma involving the mesocolon have been reported in nine patients.CASE SUMMARY The patient was a 44-year-old man with a history of pleomorphic leiomyosarcoma of the left maxilla with metastasis to the lung and liver.His most recent positron emission tomography-computed tomography(PET-CT)scan showed uptake in the ascending and transverse colons.A colonoscopy revealed a 5.0 cm×3.5 cm×3.0 cm pedunculated polyp in the ascending colon.The polyp was removed using hot snare polypectomy technique and retrieved with Rothnet.Histopathologic examination of the polyp showed a metastatic pleomorphic leiomyosarcoma.CONCLUSION Uptake(s)on PET-CT in a patient with pleomorphic leiomyosarcoma should raise suspicion for metastasis.
文摘Objective： Lung carcinoma with spindle and （or） giant cell （LCSG） is a rare epithelial malignant tumor. The aim of our study is to investigate the clinicopathological and prognostic characteristics of 17 cases of LCSGs. Methods： Among 421 patients underwent resection of lung carcinomas, 17 cases of LCSG were studied for clinical, gross and histological parameters. Follow-up information was obtained and analyzed to clarify prognostically significant parameters. Results： The LCSG patients consisted of 15 males and 2 females, with the age ranging from 45 to 78 years （median, 58 years）; 5 cases of stage Ⅰ, 3 of stage Ⅱ, 9 of stage Ⅲ by pathological TNM staging; 2 cases of exclusively spindle cell carcinoma, 5 cases of lung carcinoma with spindle cell, 10 cases of lung carcinoma with giant-cell carcinoma. Cough, chest distress, or chest pain were the most common presenting symptoms, occurring in 15 patients （88.2%）. Of 5 patients in stage Ⅰ, 4 were alive and free of relapse for more than 5 years. The difference in survival was statistically significant between LCSG and squamous cell carcinoma patients （median survival, 36 vs. 61 months; P = 0.027）. Lymph node metastasis and carcinoma with giant cell were the hazardous factors impacting postoperative prognosis of LCSG patients. Conclusion： LCSG patients in early stage may have an optimistic outcome. Lung carcinomas with giant cell displayed multiple cell components in histopathology, and poor outcome due to more lymph node involved.
文摘背景与目的:含梭形/巨细胞成分肺癌(carcinoma with spindle/giantcells,LCSG)是一种少见的恶性上皮肿瘤,相关文献报道较少。本研究目的是探讨LCSG临床病理特点及预后。方法:总结、分析17例手术治疗的LCSG患者的临床和组织病理学资料,并与同期手术的肺癌患者进行比较。利用生存曲线进行生存分析,Cox风险比例模型分析影响预后的独立因素。结果:LCSG患者男女比例为15∶2,年龄45～78岁,中位年龄58岁;pTNM分期Ⅰ期5例,Ⅱ期3例,Ⅲ期9例;病理类型梭形细胞癌2例,含梭形细胞癌(均伴一种恶性上皮成分,鳞癌3例、腺癌和大细胞癌各1例)5例,含巨细胞癌(分别有5例伴一种上皮癌、5例伴两种上皮癌)10例;4例Ⅰ期患者术后无瘤生存5年以上;LCSG患者比同期鳞癌的预后差(中位生存36个月和61个月,P=0.027),含巨细胞肺癌患者的预后比含梭形细胞肺癌(包括梭形细胞癌)患者差(中位生存18个月和64个月,P=0.026)。多因素分析显示淋巴结分期和含巨细胞癌是影响LCSG患者预后的危险因素。结论:LCSG作为一种独特类型的肺癌,其中含巨细胞的肺癌成分复杂,预后较差。
