Pleomorphic leiomyosarcoma of the adrenal gland

Primary leiomyosarcomas arising in the adrenal gland are exceedingly rare, with only 3 cases reported in the literature. We present the clinical, morphologic, and immunohistochemical features of a pleomorphic leiomyosarcoma, a variant of leiomyosarcoma that has not been described in the adrenal gland. A 63-year-old man presented with a 1-year history of enlarging right upper quadrant mass and pulmonary nodule. A diagnosis of metastatic pulmonary carcinoma to the adrenal gland was rendered on a needle biopsy specimen. Preoperative chemotherapy reduced only the pulmonary mass but not the adrenal mass, which continued to enlarge. Documented by computed tomography and confirmed at surgery, the tumor had completely replaced the right adrenal gland, invading into both the posterior aspect of the right liver and the superior pole of the right kidney. Histologic sections showed a diffuse proliferation of pleomorphic, large, and polygonal neoplastic cells with prominent nucleoli. Many bizarre mitotic figures were present. The neoplastic cells were strongly positive for desmin, calponin, and vimentin. Approximately 80% of the neoplastic cells were positive for the proliferation marker Ki-67. They were negative for smooth muscle actin, muscle-specific actin, myoglobin, myogenin, CD117, cytokeratins, carcinoembryonic antigen, epithelial membrane antigen, chromogranin, CD34, CD31, S100 protein, and HMB-45.     

Angiosarcoma of the mandibular gingiva

Angiosarcoma of the oral cavity is extremely rare. A 77-year-old woman consulted to our hospital because of polypoid mass of the mandibular gingival. Physical examination showed polypoid reddish mass measuring 1.5 × 1.5 × 1 cm in the mandibular gingival posterior to the front tooth. Enucleation of the tumor was performed. Grossly, the tumor was not encapsulated. Histologically, the tumor consisted of atypical spindle with hyperchromatic nuclei with nucleoli. Mitotic figures were scattered. Vasoformative channels were present in some areas. The surgical margins were positive. Immunohistochemically, the tumor cells were positive for factor VIII-related antigen, CD31, CD34, vimentin, p53 protein, but negative for pancytokeratin (AE1/3 and CAM5.2), S100 protein, α-smooth muscle antigen, and desmin. The Ki-67 labeling was 60%. A pathological diagnosis of angiosarcoma was made. Radical operation is planned now.     

Rhabdoid cholangiocarcinoma: a variant of cholangiocarcinoma with aggressive behavior

A rhabdoid cholangiocarcinoma is a very rare variant of sarcomatous cholangiocarcinomas. Here, we report a vimentin positive cholangiocarcinoma showing rhabdoid features in the entire tumor, with a very aggressive behavior. A 41-year-old woman was admitted to our hospital due to a huge hepatic mass. The resected liver revealed a 17 x 15 cm sized solid mass with extensive necrosis and an infiltrative border. On microscopic examination, the entire tumor was composed of loosely cohesive round to polygonal cells, with rhabdoid features having abundant eosinophilic glassy cytoplasm and eccentrically located vesicular nuclei. Some tumor cells contained intracytoplasmic mucin vacuoles, but definite areas of glandular differentiation or spindle cell were not found. Immunohistochemical staining showed a diffuse strong positive reaction to pan-cytokeratin and vimentin, and focal positivity for the carcinoembryonic antigen. Other immunohistochemical stainings for cytokeratin 7, cytokeratin 20, S-100 protein, HMB-45, desmin, alpha-smooth muscle actin, c-kit, CD34, alpha-fetoprotein, anti-hepatocyte antigen, chromogranin and synaptophysin were all negative. After two months, the patient developed a local recurrence along the resection margin, and multiple hematogenous metastases to the lung and liver were seen on the follow-up CT scan.     

Malignant myoepithelioma of the breast

Malignant myoepithelioma of the breast is rare. A 50-year-old Japanese woman was admitted to our hospital because of a right breast tumor (11 × 10 × 5.5 cm). Core needle biopsy revealed malignant spindle cells. A mastectomy was performed. The tumor consisted of malignant spindle, round, pleomorphic and giant cells with many mitotic figures and necrotic areas. Tumor and osteoclast-like giant cells were scattered. Much lymphovascular permeation was seen. In a few areas, particularly on the tumor periphery, there were merges between the tumor cells and myoepithelial cells of the non-tumorous ducts, as if the tumor emanated from the duct myoepithelium. The tumor was invasive into the skin and pectoral muscle. Immunohistochemically, the tumor cells were diffusely positive for vimentin, CD10, α-smooth muscle antigen, and Ki-67 (labeling = 95%). The significant areas of the tumor were positive for S100 protein, p63, p53, CD68, caldesmon, desmin and TGFβ1. A few areas were positive for pancytokeratin (AE1/3), cytokeratin (CK) 5/6, and CK 34βE12. In contrast, the tumor cells were negative for pancytokeratins (WSS, CAM5.2), CK7, CK8, CK14, CK18, CK19, CK20, EMA, CEA, bcl-2, myoglobin, CD34, CD56, CD45, HMB45, GFAP, α-1-antitrypsin, synaptophysin, estrogen receptor, progesterone receptor, HER2/neu, MUC1, MUC2, MUC5AC and MUC6. The author diagnosed the tumor as malignant myoepithelioma, as myoepithelial markers (C10, p63, S100 protein, α-smooth muscle actin, caldesmon) were positive, and also because there was a transition between the tumor cells and myoepithelium of non-tumorous ducts. The grade of the tumor was high. The patient was treated with chemoradiation and was free of disease 5 months after the operation.     

Vascular leiomyoma of the lung arising from pulmonary artery

Leiomyoma of the lung is extremely rare. The entity is not described in WHO blue book. Less than 100 cases of leiomyoma of the lung have been reported in the literature. However, vascular leiomyoma has not been reported in the literature, to the author’s best knowledge. Herein reported is the first case of vascular leiomyoma of the lung arising from smooth muscles of the pulmonary artery. A 62-year-old woman (non-smoker) was found to have a small tumor in the upper lobe in the right lung in routine check. Imaging modalities including CT demonstrated no metastatic lesions. Although clinical cytology and biopsy revealed no malignant cell, right upper lobectomy was performed under the clinical diagnosis of lung carcinoma. Grossly, a white tumor of 1 x 0.8 cm was recognized in the lung. Microscopically, the tumor was connected to the pulmonary arteries. The tumor was composed of mature smooth muscles. Small pulmonary arteries are embedded in the tumor. No lymphatics were seen. Immunohistochemically, the tumor cells were poisitive for alpha-smooth muscle actin, vimentin and Ki-67 (labeling 2%). However, they were negative for cytokeratin (CK) AE1/3, CK CAM5.2, desmin, S100 protein, p53, CD34, KIT, HMB45, estrogen receptor, progesterone receptor, and myoglobin. A pathological diagnosis of primary vascular leiomyoma arising from the smooth muscle of pulmonary artery was made. The patient is now free from tumor, and is now alive 10 year after the operation.     

间叶性软骨肉瘤23例临床病理和免疫表型分析

目的 探讨间叶性软骨肉瘤的临床病理和免疫表型特点.方法 复习23例骨及软组织间叶性软骨肉瘤的临床和病理学资料,对其中14例进行免疫组织化学(EnVision法)染色.结果男性14例,女性9例,年龄范围12～47岁,其中原发于骨的病变13例,原发于软组织5例,早期症状以局部肿块和疼痛为主.镜下组织学特点为含有原始间叶性小细胞和分化良好的软骨成分,二者有移行过渡,可见"骺板样软骨".原始间叶性小细胞免疫组织化学染色阳性表达Sox9(14/14)、CD99(12/14)、波形蛋白(6/14)、CD56(4/14)、CD57(4/14)、神经元特异性烯醇化酶(3/14)、结蛋白(1/14);Ⅱ型胶原、S-100蛋白、上皮细胞膜抗原、PCK、突触素、嗜铬粒素A、CD34、c-erbB2均阴性.结论间叶性软骨肉瘤少见,结合组织病理形态特征及免疫组织化学染色对病理鉴别诊断有较大帮助.

Abstract：

Objective To study the clinicopathologic and immunohistochemical features of mesenchymal chondrosarcoma. Methods The clinical and histologic features of 23 cases of mesenchymal chondrosarcoma were analyzed. Immunohistochemical study was also performed in 14 of the cases. ResultsThe age of patients ranged from 12 to 47 years. Fourteen of them occurred in males. Thirteen cases involved the bony skeleton and 5 cases affected the soft tissue. The patients presented with pain and/or swelling. Histologically, the tumor consisted of a mixture of undifferentiated small round cells and hyaline cartilage. Transition between the two components was demonstrated and growth plate-like cartilage was observed. Immunohistochemical study showed that the small round cells were positive for Sox9 (14/14), CD99(12/14), vimentin (6/14), CD56 (4/14), CD57(4/14), neuron-specific enolase (3/14) and desmin(1/14). They were negative for Coll-Ⅱ, S-100 protein, epithelial membrane antigen, pan-cytokeratin, synaptophysin, chromogranin A, CD34 and c-erbB2. Conclusions Mesenchymal chondrosarcoma is a rare malignant tumor. Thorough histologic examination, when coupled with immunohistochemical findings, is helpful in arriving at a correct diagnosis.     

头皮低分化皮肤血管肉瘤的临床病理分析

目的 探讨头皮低分化皮肤血管肉瘤的临床免疫病理特征.方法 分析8例头皮低分化皮肤血管肉瘤患者的临床特点,观察其组织病理形态和CD31、CD34、第八因子相关抗原、波形蛋白、细胞角蛋白(AEl/AE3和CAM5.2)、上皮细胞膜抗原(EMA)、癌胚抗原(CEA)和S-100蛋白免疫组织化学染色.结果 患者平均发病年龄为69.0岁,男:女为5:3,临床早期常在头面部出现暗红斑,后期发生浸润性暗红斑块,均伴结节,偶发溃疡.组织病理检查示真皮广泛梭形细胞局部或广泛实性浸润,常见细胞异形,未见明确血管腔样结构.梭形细胞强阳性表达CD31、第八因子相关抗原和波形蛋白,弱阳性表达CD34、AEl/AE3和CAM5.2,而EMA、CEA和S-100蛋白阴性.结论 头面部出现淤斑样浸润性斑块和结节的老年患者,应及时做组织病理检查,CD31、CD34、第八因子相关抗原和波形蛋白免疫组织化学标记有助于明确诊断.     

Malignant stromal tumor of the gallbladder with interstitial cells of Cajal phenotype

Malignant mesenchymal tumors of the gallbladder are exceedingly rare. We report a malignant stromal tumor of the gallbladder with a phenotype of interstitial cells of Cajal. To our knowledge, only the benign counterpart of this tumor has been described previously. A 34-year-old woman presented with right upper quadrant abdominal pain. At the time of cholecystectomy, the gallbladder was noted to have a thickened wall and a polypoid mass arising in the neck of the gallbladder. Histologic sections showed a cellular proliferation of spindle neoplastic cells that were arranged in short fascicles. Numerous mitotic figures and foci of necrosis were noted. The neoplastic cells expressed CD117 (c-Kit protein) and vimentin. They were negative for smooth muscle actin, desmin, myoglobin, cytokeratin, S100 protein, and CD34. Our case demonstrates that a malignant stromal tumor that is histologically and immunohistochemically identical to gastrointestinal stromal tumor can occur in the gallbladder, and that the expression of CD117 may be of therapeutic importance.     

Solitary fibrous pseudopapillary tumor of the lung: pulmonary fibroadenoma and adenofibroma revisited

We describe a peculiar pulmonary lesion, that we interpreted as a pseudopapillary variant of solitary fibrous tumor. The patient was a 62-year-old asymptomatic male, non smoking, presenting with a peripheral nodule, 0.8 cm across, located in the lower lobe of the right lung. The patient is alive and well 18 months after surgical excision of the nodule. Microscopically, the lesion was well-circumscribed and characterized by a diffuse pseudopapillary pattern. Pseudopapillae were large, and were covered by a rim of cubic epithelium devoid of atypia. The stromal axis was fibrous and contained scattered bland spindle cells. Immunohistochemically, the latter were strongly positive for vimentin and CD34, focally positive for BCL2 and CD99, negative for cytokeratin, EMA, TTF1, calretinin, smooth muscle actin, desmin and S100 protein; the epithelial cells were immunoreactive for cytokeratin, EMA and TTF1. We interpret this lesion as a peculiar pseudopapillary variant of solitary fibrous tumor, corresponding to what has been reported in the literature as pulmonary adenofibroma and fibroadenoma. The most important differential diagnostic considerations are briefly discussed.     

脾脏炎性假瘤样滤泡树突细胞肿瘤的临床病理学特征

目的探讨炎性假瘤样滤泡树突细胞肿瘤的临床病理学特征。方法观察1例脾脏的炎性假瘤样滤泡树突细胞肿瘤的大体和光镜形态,免疫组织化学EnVision法检测CD21、CD23、CD35、Clusterin、S-100、波形蛋白、平滑肌肌动蛋白（SMA）、CD1a、CD68、间变型淋巴瘤激酶（ALK）、CD30、CD31、CD34、CD3、CD20,原位杂交检测EBER,并结合文献复习分析其生物学行为和病因机制。结果炎性假瘤样滤泡树突细胞肿瘤好发于腹腔内脏器,尤以肝、脾多见,患者以女性为主。一般肿瘤较大,切面灰白质韧,中心有出血或坏死。镜下大量淋巴细胞、浆细胞背景中见弥散分布的滤泡树突细胞,形似炎性假瘤。高倍镜下滤泡树突细胞胞质淡染,胞界不清,核卵圆形或杆状,染色质空泡样或点彩状,居中可见小的紫色核仁。部分瘤细胞显示异型性,核膜皱褶,染色质粗糙,核仁明显。核分裂象罕见。免疫组织化学：梭形瘤细胞波形蛋白强阳性;Clusterin、SMA、CD68均阳性;CD35局部弱阳性,S-100弱阳性;CD21、CD23、CD1a、ALK、CD30、CD31、CD34均阴性。背景淋巴细胞CD3阳性,灶性区CD20阳性。原位杂交：梭形瘤细胞EBER阳性。结论炎性假瘤样滤泡树突细胞肿瘤是一种罕见的低度恶性肿瘤,与EB病毒感染有关。     

Proximal-type epithelioid sarcoma: report of two cases in the perineum: differential diagnosis and review of soft tissue tumors with epithelioid and/or rhabdoid features.

The authors report two cases of perineal proximal-type epithelioid sarcoma in middle-aged men, age 51 and 43 years old. Both tumors were located in the right side. In the first patient a 7.5-cm, well-encapsulated tumor was completely excised. The second patient was a referral case with incomplete excision, but the computed tomography scan and magnetic resonance imaging showed a 14-cm nonencapsulated tumor involving the soft tissues of the inner thigh and perineum, as well as metastasis in right inguinal and retroperitoneal lymph nodes. Both neoplasms had a predominant solid pattern alternating with occasional discohesive areas. Both were composed of large oval to polygonal cells with vesicular nuclei, conspicuous nucleoli, and amphophilic to eosinophilic cytoplasm. Rhabdoid phenotype was identified in the second case only. The first neoplasm displayed 15% necrosis, 7 mitoses per 10 high-power field, focal vascular invasion, and no extracapsular invasion. The other exhibited 60% necrosis, 12 mitoses per 10 high-power fields, extensive vascular invasion, no distinct capsule, and invasion of the surrounding fatty tissue. Both were positive for vimentin, cytokeratin, epithelial membrane antigen, and CD34. Muscle-specific actin was negative in the first case and focally positive in the second. CD56 was positive in the second case and negative in the first case. Desmin, CD45, CD30, factor VIII, CD31, S100, HMB45, calretinin, and synaptophysin were negative in both. Since proximal-type epithelioid sarcoma can be confused with a number of other soft tissue tumors with epithelioid and/or rhabdoid features, the authors emphasize the immunohistochemical differential diagnosis.     

血管瘤样纤维组织细胞瘤病理诊断与鉴别诊断

目的 探讨血管瘤样纤维组织细胞瘤(angiomatoid fibrous histiocytoma)的临床病理特点、免疫表型、病理诊断与鉴别诊断要点.方法 收集5例血管瘤样纤维组织细胞瘤,分析其临床特点,观察病理组织学形态及免疫表型特征,并复习相关文献.结果 临床特点:5例患者中男性3例,女性2例,平均年龄21.4岁,平均病程约13个月.主要表现为真皮深层或皮下缓慢生长的无痛性肿块,3例位于头颈部,1例位于肘部,1例位于足部,均行完整切除,术后未予放疗和化疗等辅助治疗,随访10～29个月,无一例复发和转移.病理检查:5例均为单发肿块,瘤体最大径平均1.9 cm,周界清楚,切面呈灰褐色,质实,较硬,有不规则出血性囊腔.镜下观察:瘤细胞主要呈组织细胞样、梭形,略呈结节状分布,5例均有假血管样腔隙,3例有较厚纤维性假包膜及周围淋巴细胞浆细胞浸润.免疫组织化学:5例均表达波形蛋白,3例表达结蛋白,3例表达CD68,2例表达上皮细胞膜抗原,2例表达CD99,5例均不表达S-100蛋白、CKpan、CD34和CD31.结论 血管瘤样纤维组织细胞瘤是一种罕见的交界性肿瘤,其诊断主要依靠病理形态学特征,免疫组织化学标记有助于诊断和鉴别诊断;局部适当扩大切除,术后随访患者是主要处理原则.     

Lymphangioma and congenital pulmonary lymphangiectasis: a histologic, immunohistochemical, and clinicopathologic comparison.

Lymphangioma (LA) and congenital pulmonary lymphangiectasis (CPL) are part of a spectrum of lymphatic disorders less well characterized than other vascular tumors and malformations. Recent studies showed proliferative and involutional growth phases for hemangiomas that distinguish them from malformations. We investigated immunohistochemical reactivity and proliferative activity to determine whether a similar diagnostically/prognostically useful pattern exists for LA, comparing LA with CPL as a malformative lesion. Immunohistochemical tests for vimentin, Factor VIII-related protein, CD31, CD34, CD45RO, smooth muscle actin, Type IV collagen, MIB-1, bcl-2, and topoisomerase IIalpha were performed on 20 LAs and 10 cases of CPL. Giemsa staining was also performed to quantitate mast cells. Clinicopathologic correlation was performed by medical record review. LA and CPL shared a similar immunohistochemical profile for vimentin, Factor VIII-related protein, CD31, CD34, smooth muscle actin, CD34, and, to a lesser extent, CD45RO. CD31 and CD34 displayed the most uniform pattern of endothelial reactivity, although CD34 had high background staining. bcl-2 was negative. Four LAs exhibited focal low reactivity for MIB-1 and topoisomerase IIalpha; recent infection and thrombosis were associated conditions. LAs displayed seven-fold more mast cells and more reactive T lymphocytes than did cases of CPL. LA and CPL had similar immunohistochemical profiles; LA resembled vascular malformations more than hemangiomas. CD31 and CD34 were useful for detection of small lymphatics at resection margins of LA, a feature associated with recurrence. MIB-1 and topoisomerase IIalpha expression were associated with inflammatory, thrombotic, or reactive processes and were not diagnostically useful. Abundant mast cells, which also were noted in other soft tissue neoplasms, prompt speculation concerning their role in the growth of LAs.     

Epulis-like gingival angiosarcoma of the mandible: a case report

A 55-year-old woman consulted our hospital for an epulis-like small mass in the anterior region of the mandible. A biopsy of the tumor was performed. Histological analysis showed that the tumor consisted of spindle-shaped and polygonal cells with hyperchromatic nuclei, and intracytoplasmic vacuoles and mitotic figures were scattered. Immunohistochemical staining revealed that the tumor cells were positive for factor VIII-related antigen, CD31, αSMA, and vimentin, but negative for pancytokeratins, S100 protein, neuron-specific enolase, and CD56. The Ki-67 labeling index was more than 50%. Based on these findings, a final pathological diagnosis of angiosarcoma was made. The tumor did not invade into the surrounding tissue. The operation was performed with about a 20-mm surgical margin that was negative for tumor invasion. After a 4-year follow-up, no metastatic lesions were found, and the primary site was covered with a partial denture.     

An immunohistochemical study of metaplastic spindle cell carcinoma, phyllodes tumor and fibromatosis of the breast

The diagnosis of metaplastic (sarcomatoid) carcinoma (MSC) of breast often requires immunohistochemistry with a cytokeratin (CK) panel to distinguish them from phyllodes tumors (PT), primary sarcomas, and fibromatoses. CK staining may be heterogeneous in metaplastic carcinomas. The aim of the study was to investigate the theory that MSCs show evidence of myoepithelial differentiation and to evaluate immunohistochemical markers that may be helpful in distinguishing MSCs from PT and fibromatosis. We reviewed histology and performed immunohistochemistry for AE1/AE3, 34betaE12, CK5 and CK14, Cam5.2, CK7 and CK19, epithelial membrane antigen (EMA) (B55), smooth muscle actin (SMA), S100, desmin, vimentin, CD31, CD34, and bcl-2 on paraffin-embedded tissue from 18 MSCs, 26 PTs, and 8 fibromatoses. We assessed staining by using a semiquantitative method. Sarcomatous areas in MSCs were positive for 34betaE12 in 11 cases; for SMA in 10; for CK5 in 7; for CK14 in 6; for Cam5.2, AE1/AE3, and S100 in 5; and for CK7 and CK19 in 3. No CK expression was seen in stromal areas in PT or in fibromatoses. CD34 and bcl-2 were more frequently expressed in spindle cell areas in PTs (18 and 12 of 26, respectively) than in MSCs (0 and 2 of 18, respectively). MSCs show strong evidence of myoepithelial differentiation. CD34 and, to a lesser extent, bcl-2 positivity in PTs may be helpful in differentiating these two lesions from MSCs, particularly in small biopsies, because CK staining in MSCs may be heterogeneous. In our hands, 34betaE12 was the CK most frequently expressed in sarcomatoid areas in MSCs.     

Chordoid glioma: a case report and molecular characterization of five cases

Chordoid gliomas are rare, slow-growing neoplasms of the anterior third ventricle. We reported a case of chordoid glioma in a 41-year-old man with obstructive hydrocephalus. Histologically, the tumor consisted of polygonal epithelioid cells admixed with elongated cells in a myxoid stroma. A prominent lymphoplasmacytic infiltrate was present. The tumor cells expressed glial fibrillary acidic protein (GFAP), epithelial membrane antigen (EMA), vimentin, CD31, CD34, epidermal growth factor receptor (EGFR) and S100 but were negative for pankeratin and E-cadherin. The percentage of Ki67 positive cells was approximately 3%. Weak p53 immunoreactivity was seen in less than 10% of the cells. Array comparative genomic hybridization performed on this case, as well as on four other archived cases, showed losses at several loci. Fluorescence in situ hybridization (FISH) confirmed consistent genetic alterations at 9p21 and 11q13. These are the fifth through ninth reported cases of chordoid gliomas with molecular characterization suggesting a distinct genetic origin from other gliomas.     

Sarcomatoid carcinoma in the pelvic cavity

Sarcomatoid carcinoma in the pelvic cavity is very rare. A 58-year-old Japanese man was admitted to our hospital because of lower abdominal fullness. CT and MRI revealed a large mass in the left pelvic cavity. Transurethral bladder endoscopy showed tumor invasion, and large biopsies were obtained from the bladder lesion. Histologically, the tumor was composed of malignant round cells with hyperchromatic nuclei. Many intracytoplasmic vacuoles were present. No carcinomatous areas were seen. Immunohistochemically, the tumor cells were positive for cytokeratin (CK) 18, vimentin, p53 and Ki-67 (labeling 80%). The tumor cells were negative for panCK AE1/3, CD5/6, CK7, CK8, CK14, CK19, CK20, CK 34BE12, EMA, desmin, calretinin, WT-1, S100 protein, α-smooth muscle actin, CEA, CD34, CD45, CD20, factor VIII-related antigen, synaptophysin, p63, CDX2, and myoglobin. Because the CK18 was diffusely expressed, the pathological diagnosis was sarcomatoid carcinoma.     

Fatal poorly differentiated angiosarcoma of the scalp

Cutaneous angiosarcoma is a very rare but aggressive tumor. Angiosarcoma of the scalp is very rare, and a review of the world literature revealed less than 60 cases. Here, the author reports a case of poorly differentiated angiosarcoma of the scalp. The pathological diagnosis was very difficult. A 70-year-old Japanase man was admitted to our hospital complaining of red mass of the scalp. An excisional biopsy was done. The biopsy showed proliferation of malignant spindle cells in the dermis. Apparent differentiation was not recognized. Invasion into the lateral dermis and subcutis was recognized. There were many mitotic figures and a few foci of necrosis. The size was 2 × 2 × 3 cm. Intracytoplasmic vacuoles were recognized in the malignant tumor cells in some places. A few vague vasoformative features were recognized in one very small area. Immunohistochemically, the malignant spindle cells were positive for factor VIII-related antigen (F-VIII-RA), Ulex lectin, CD31, CD34, vimentin, p53 protein. The Ki-67 labeling was 76%. In contrast, the tumor cells were negative for cytokeratins, epithelial membrane antigen, desmin, S100 protein, α-smooth muscle antigen, bcl-2, melanosome, and myoglobin. The intracytoplasmic vacuoles were strongly positive for F-VIII-RA, Ulex lectin, CD31, and CD34, The abortive vasoformative channels were moderately positive for these endothelial markers. A pathologic diagnosis of angiosarcoma of the scalp was made. Chemoradiation and immunotherapy were performed. However, the tumor recurred several times, and ultimately metastasized to the systemic bones and lungs. The patient died of systemic carcinomatosis 33 months after the first manifestation.     

Epithelioid angiosarcoma of the kidney: a diagnostic dilemma in fine-needle aspiration cytology

Epithelioid angiosarcomas (EAS) of kidney are rare and aggressive tumors with noncharacteristic imaging features that overlap with those of inflammatory conditions and renal cell carcinoma (RCC). We report the fine-needle aspiration (FNA) cytology findings of a case of EAS that involved the left kidney of an 83-year-old male. The smears and cell block sections showed pleomorphic epithelioid cells with ample cytoplasm, eccentric nuclei, occasional cytoplasmic hyaline globules, and rare intracytoplasmic lumina. Immunohistochemical stains performed on cellblock sections showed that the tumor cells were positive for vimentin, CD31, CD34, Factor VIII, and CD10, but showed no staining for cytokeratins AE1/AE3 and 8/18, HMB45, CD45, smooth muscle actin, and S100. The morphological and immunohistochemical distinction between EAS, primary tumors of the kidney, and other epithelioid and rhabdoid neoplasms is discussed.     

肢端黏液炎性纤维母细胞性肉瘤

目的 探讨肢端黏液炎性纤维母细胞性肉瘤的临床病理学特征、免疫学表型及其鉴别诊断。方法 对1例发生于足背和右小腿远端的肢端黏液炎性纤维母细胞性肉瘤进行光镜观察和免疫组化标记。结果 患者因足背皮下“结节性筋膜炎”局部切除术后复发就诊。体检发现足背至右小腿远端前外侧皮下多发性结节,直径1～4cm,影像学检查提示肿瘤累及深部骨膜。镜下肿瘤由比例不等的黏液样区、透明变性区及炎症性区域混合组成。黏液样区域主要由交织条柬状排列的梭形瘤细胞组成,核显示轻至中度异型性,核分裂象罕见,间质疏松、黏液样,局部区域可见黏液湖形成。其内可见单空泡印戒样或多空泡状假脂肪母细胞,形态类似黏液纤维肉瘤。透明样区域由散在的胖梭形至卵圆形的瘤细胞和透明样间质混和组成。炎症性区域由成簇的淋巴细胞组成,与黏液样区域和透明变性区相混杂。病变内可见体积较大类似节细胞或R-S细胞的畸形细胞。免疫组化标记显示瘤细胞弥漫表达Vim,个别细胞表达p53,而CD68、actin、Des、CD34、CD30和S-100蛋白等标记均为阴性,多数淋巴细胞表达CD45RO。结论 肢端黏液炎性纤维母细胞性肉瘤是一种罕见的低度恶性软组织肉瘤,瘤细胞由变异的纤维母细胞衍化而来,熟悉其形态学特征对避免误诊为其它良性或恶性病变具有重要意义。该瘤常在局部呈侵袭性生长,具有较高的复发率,临床上应予以完整切除。