Endometrial stromal neoplasms of the uterus. A clinicopathologic study.

A clinicopathologic study of uterine endometrial stromal tumors (EST) has been performed with special emphasis on histologic and immunohistochemical differential criteria and prognostic factors. The material comprised three stromal nodules (SN), twelve low grade stromal sarcomas (LGESS) and five high grade stromal sarcomas (HGESS). Previously unreported endolymphatic growth was found within one SN. EST showed an association of mitotic index (IM) with atypia, degree of stromal differentiation, additional non-stromal differentiation and venous invasion. IM was the best criterion in the differential diagnosis of LGESS and HGESS and the most significant histologic prognosticator. The present study shows that a histologic grade of stromal sarcoma was a more significant prognostic factor than pTNM stage. The results suggested that clinicopathologic classification of EST could be supplemented by including the following subgroups: a) SN--without intravascular growth, and potentially malignant SN--with endolymphatic growth within the tumor; b) LGESS with IM < 2 and no atypia, and LGESS with 2 > IM < 10 and mild atypia; c) HGESS with 10 > IM < 20 and moderate atypia, and HGESS with IM > 20 and marked atypia. Contrary to common view these observations indicate that the distinction of some SN and LGESS from stromal hyperplasia is possible in an endometrial curretage material.     

Follicular dendritic cell sarcoma presenting as a submucosal tumor of the stomach

Follicular dendritic cell (FDC) sarcomas, especially those of extranodal origin, are extremely rare, and this entity could easily be missed without a high index of suspicion. We report a case of FDC sarcoma presenting as a submucosal tumor of the stomach in a 45-year-old man. The mass was a spindle and epithelioid mesenchymal tumor with many individually scattered and perivascular aggregates of lymphocytes. Immunohistochemical and ultrastructural studies confirmed the diagnosis. Although more than 50 cases of this tumor have been documented in the English literature, to our knowledge the presentation of FDC sarcoma as a submucosal tumor of the stomach has never been recorded. This case highlights the occurrence of FDC sarcoma as a submucosal tumor of the gastrointestinal tract. We believe that FDC sarcoma should be included in the differential diagnosis of spindle or epithelioid cell tumors of the gastrointestinal hollow viscus to prevent this still under-recognized tumor from being overlooked.     

子宫内膜间质肉瘤9例临床病理分析

目的 探讨子宫内膜间质肉瘤(endometrial stromal sarcoma,ESS)的临床病理特征、诊断、鉴别诊断及预后.方法 对9例ESS患者进行临床、病理资料分析、免疫组化检测及随访.结果 患者年龄39～64岁,中位46.3岁.临床主要表现为阴道流血及子宫增大/占位.肿瘤直径2.3～11 cm,平均4.6 cm.光镜下8例呈低度恶性子宫内膜间质肉瘤(low grade endometrial stromal sarcoma,LGESS),均由类似增殖期子宫内膜间质肿瘤细胞构成,细胞密集,异型性不明显,呈不规则舌状或岛状浸润肌层,并伴较多薄壁螺旋小血管;1例为高度恶性子宫内膜间质肉瘤/未分化子宫内膜肉瘤(high grade endometrial stromal sarcoma/undifferentiated endometrial sarcoma,HGESS/UES),肿瘤细胞直接替代子宫肌层,具有明显的细胞异型性,无LGESS常见的螺旋小血管.免疫组化检测显示肿瘤细胞CD10、vimentin均阳性,PR、ER大部分阳性,SMA和desmin及h-Caldesmon为极少数局灶阳性,S-100、CD34均阴性.术后随访7例(平均53个月),只有1例HGESS/UES死亡.结论 ESS是女性生殖道很少见的一种恶性肿瘤,恶性度相差很大.确诊主要依靠其临床病理特点,并辅以免疫组化标记综合分析.诊断时要与子宫内膜间质结节、平滑肌肿瘤、低分化癌等鉴别.     

Spindle cell tumors of the mediastinum

Spindle cell tumors originating in the mediastinum are extremely rare. Due to the profusion of structures and organs located in the mediastinum, a wide variety of spindle cell neoplastic processes can develop in this location. These include various different tumor types including epithelial, vascular, lipomatous, fibroblastic and neural tumors among others. Many of these different tumor types are associated with specific immunohistochemical and molecular genetic profiles that help differentiate them from each other. Although spindle cell morphology has traditionally been associated with mesenchymal neoplasms, in the mediastinum the most common spindle cell tumor is spindle cell thymoma, an epithelial rather than mesenchymal neoplasm. Except for neurogenic tumors originating in the posterior mediastinum, mesenchymal neoplasms are quite rare in mediastinal locations and require clinical correlation to rule out the possibility of a metastasis from an extra-mediastinal soft tissue or somatic sarcoma. Herein we will review the most common types of spindle cell neoplasms that occur in the mediastinum, with particular emphasis in their differential diagnosis and the role of ancillary techniques for diagnosis.     

Sarkom der Milz mit MDM2-Expression

Primary sarcomas and sarcoma metastases are a rarity in the spleen. We report on the case of a 69-year-old male patient presenting with unclear abdominal symptoms and computed tomography (CT) revealed a tumor mass in the spleen. Histologically the tumor mass predominantly showed features of a spindle cell sarcoma with lymphoid infiltrates. The expression and amplification of MDM2 could be demonstrated by means of immunohistochemistry and fluorescence in situ hybridization (FISH). Furthermore, staging examinations did not reveal indications of any other primary tumors. These preliminary findings were suggestive of a dedifferentiated liposarcoma; however, in the further diagnostic work-up the tumor showed strong expression of CD21 and CD23 and was ultimately diagnosed as a follicular dendritic cell sarcoma (FDCS). The case emphasizes that MDM2 expression represents a possible pitfall in the diagnosis of spindle cell tumors. The differential diagnostic distinction between FDCS and a dedifferentiated liposarcoma is discussed.     

Podoplanin (D2-40) is a novel marker for follicular dendritic cell tumors

Podoplanin, recognized by monoclonal antibody D2-40, may be a useful marker for follicular dendritic cell (FDC) tumors. Paraffin sections of 125 dendritic cell, histiocytic, and spindle cell lesions were studied, including 11 FDC tumors, 5 interdigitating dendritic cell tumors, 10 histiocytic sarcomas, 5 Langerhans cell histiocytosis, 5 sinus histiocytosis with massive lymphadenopathy, 5 inflammatory pseudotumors of lymph node or spleen, 9 nodal Kaposi sarcomas, 6 inflammatory myofibroblastic tumors (IMTs), 29 gastrointestinal stromal tumors (GISTs), and 10 cases each of malignant peripheral nerve sheath tumor, leiomyosarcoma, monophasic synovial sarcoma (SS), and solitary fibrous tumor. All FDC tumors and Kaposi sarcomas showed strong immunoreactivity for podoplanin (predominantly membranous). Podoplanin expression was only occasionally observed in the other tumor types, including 7 GISTs (24%), 2 IMTs (33%), and 3 SS (30%), and was generally weak and cytoplasmic. Reactivity for podoplanin was more common in spindle cell GISTs (5/13 [38%]) than in epithelioid or mixed-type GISTs (2/16 [13%]). Podoplanin is a highly sensitive marker for FDC tumors and may be useful to help confirm the diagnosis in conjunction with conventional FDC markers, particularly in the differential diagnosis of dendritic cell and histiocytic lesions.     

Synovial sarcoma presenting as a lung mass and diagnosed by cytology

Synovial sarcoma is a common soft tissue sarcoma with variable fibrous and epithelial differentiation that rarely arises from other body sites, such as within the lung. A case of a 68‐year‐old male with an extensive smoking history who presented with chest pain and a primary, central, metabolically active lung mass was reported. The mass was biopsied by bronchial brushing, bronchoalveolar lavage (BAL), and transbronchial fine‐needle aspiration (FNA). Cytologic analysis of bronchial brushing, BAL, and FNA revealed single and clusters of atypical spindle cells, oval, or spindle‐shaped nuclei with smooth nuclear membranes, hyperchromatic and granular chromatin, scant to moderate and delicate cytoplasm, a high degree of mitotic figures, and a lack of necrosis. Immunohistochemical studies showed that the tumor cells were positive for CD99, BCL2, and CK7. A diagnosis of synovial sarcoma was rendered. The differential diagnosis of primary pulmonary synovial sarcoma is discussed, including neuroendocrine tumors, squamous cell carcinoma, and various sarcomatous tumors. Diagn. Cytopathol. 2016;44:434–437. © 2016 Wiley Periodicals, Inc.     

Undifferentiated sarcoma of the parotid gland with osseous metaplasia

Malignant spindle cell tumors of the parotid gland are a diagnostic challenge. We present an unusual case of such a tumor that occurred in the right parotid gland of a 53-year-old man. The clinical and histologic assessments were consistent with a primary sarcoma of the parotid gland. The tumor was composed of sheets of pleomorphic, spindle-shaped cells with an area of bone formation. By immunohistochemistry, the tumor cells were positive for vimentin and negative for epithelial markers. Electron microscopy revealed mesenchymal cells containing moderate amounts of rough endoplasmic reticulum. The major differential diagnostic considerations were spindle cell carcinoma, carcinosarcoma, and primary undifferentiated sarcoma with osseous metaplasia. The lack of epithelial features and the benign appearance of the bone formation led to a diagnosis of undifferentiated sarcoma of the parotid gland.     

Primary stromal sarcoma of breast with malignant fibrous histiocytoma‐like features causing diagnostic dilemma on fine‐needle aspiration cytology in a patient with squamous cell carcinoma of cervix: A case report

A variety of malignant spindle‐cell tumors are known to occur in the breast, which are collectively referred to as “breast spindle‐cell tumors.” Their interpretation on fine‐needle aspiration cytology (FNAC) can be highly tricky. Here, we report a case. A 47‐year‐old female presented with a hard, mobile lump in the right breast for 4 months. Five years previous to this, she was treated with radiotherapy for squamous‐cell carcinoma (SCC) of cervix. FNA of the breast lump showed high‐cell yield with clusters, fascicles, and dissociated cells displaying spindle‐cell morphology, with marked pleomorphism, many bizarre uninucleate/multinucleate giant cells, and a striking mitotic activity. At places, the cells in a necrotic background simulated keratinized squamous cells. Considering the past history of SCC of cervix, the first possibility of metastatic SCC with a differential diagnosis of metaplastic carcinoma, malignant phylloides, and a primary sarcoma of the breast was considered. Subsequent trucut/excision biopsy with appropriate immunomarkers revealed it to be a case of primary stromal sarcoma ofthe breast with malignant fibrous histiocytoma (MFH)‐like features. Malignant spindle‐cell tumor of breast in a patient with SCC of cervix (which also has a tendency to manifest with spindle cell morphology) can cause genuine diagnostic difficulty. In such patients, use of appropriate panel of markers is mandatory for distinguishing between the metastatic SCC from cervix and other more commonly occurring spindle‐cell malignancies of the breast. Diagn. Cytopathol.2011;39:223–228. © 2010 Wiley‐Liss, Inc.     

Diagnostic pitfalls associated with fine-needle aspiration biopsy in a patient with the myxoid variant of monophasic fibrous synovial sarcoma

Synovial sarcoma (SS) is one of the most common soft tissue tumors that typically presents in the extremities of young adults, but may occur at any site and affect children during the first decade. Herein we discuss a 12-yr-old male who complained of left foot pain and plantar mass. A fine-needle aspiration biopsy of an 8 cm subcutaneous mass was performed revealing a myxoid spindle cell neoplasm. The cytologic differential diagnosis included a myxoid neurofibroma, neurothekeoma, and a myxoid sarcoma. Subsequent excision of the mass revealed a monophasic fibrous SS with myxoid features. Examination of the tissue by fluorescence in situ hybridization confirmed the presence of characteristic SS SYT gene rearrangement at chromosome 18q11.2. This case underscores that the cytologic distinction of mxyoid spindle cell tumors may be challenging. We report the cytologic features of a myxoid monophasic fibrous SS, and discuss its distinction from other benign and malignant myxoid soft tissue neoplasms.     

p63 immunohistochemical staining is limited in soft tissue tumors

p63 is a p53 homolog that is expressed in various normal epithelial tissues and epithelial malignancies. Its expression in mesenchymal lesions has not been examined in depth; therefore, we studied p63 expression by immunohistochemical analysis in 650 soft tissue tumors. We found that p63 expression is limited in soft tissue tumors. The majority of tumors studied were p63-, including all cases of angiosarcoma, lipomatous neoplasms, dermatofibrosarcoma protuberans, solitary fibrous tumor, schwannoma, neurofibroma, gastrointestinal stromal tumor, and leiomyosarcoma. Nuclear p63 reactivity was found in a subset of soft tissue myoepithelioma and myoepithelial carcinoma of soft tissue, cellular neurothekeoma, soft tissue perineurioma, Ewing sarcoma/peripheral neuroectodermal tumor, diffuse-type giant cell tumor, and giant cell tumor of soft parts. Infrequent, weak, or focal p63-staining patterns were observed in low-grade fibromyxoid sarcoma, malignant peripheral nerve sheath tumor, extraskeletal myxoid chondrosarcoma, myxofibrosarcoma, proximal-type epithelioid sarcoma, synovial sarcoma, embryonal rhabdomyosarcoma, desmoplastic small round cell tumor, atypical fibroxanthoma, and spindle cell melanoma. Absent p63 expression is typical for most soft tissue tumors, including most (but not all) that would be in the differential diagnosis of spindle cell squamous carcinoma.     

Spindle cell lesions of the adult prostate.

Prostatic spindle cell lesions are diagnostically challenging and encompass a broad array of benign and malignant processes. A subset of these lesions arises only within the prostate and generally represents entities that originate from the prostate epithelium or stroma, such as sclerosing adenosis, sarcomatoid carcinoma, stromal tumors of uncertain malignant potential (STUMP), and stromal sarcoma. Another subset of spindle cell tumors that involve the prostate are also found at other sites and include solitary fibrous tumor, leiomyosarcoma, and neural lesions among others. Finally, tumors may secondarily involve the prostate yet present as primary prostatic processes, as is evident with several cases of gastrointestinal stromal tumors (GIST). The utility of ancillary studies, including immunohistochemistry, is often limited and the main criteria for diagnosis are the morphologic findings by routine H&E stain. This review addresses the various entities that may present as spindle cell tumors within the adult prostate and discusses the functional aspects of the differential diagnosis of these lesions.     

原发性肾脏滑膜肉瘤临床病理及分子遗传学分析

目的探讨原发性肾脏滑膜肉瘤的临床病理特征、诊断及鉴别诊断。方法对1例罕见的原发性肾脏滑膜肉瘤进行光镜观察和免疫组化染色,并用RT-PCR方法在石蜡包埋组织中检测SYT-SSX融合mRNA表达,结合文献分析讨论。结果肿瘤由核分裂活跃的单形的梭形细胞组成,呈交织的束状或实性排列,部分区域呈血管外皮瘤样图像,未见上皮分化成分;囊性区囊内衬核分裂不活跃的多边形的鞋钉样上皮。免疫表型瘤细胞vimentin、CD99、bcl-2、S-100均阳性,Ki-67强阳性;CK(AE1/AE3)、EMA、SMA、CD117、CD34、p53、ER、PR均阴性。RT-PCR方法检测到肿瘤组织中有SYT-SSXmRNA表达,对照组SYT-SSX无表达。患者术后5月内死亡。结论原发性肾脏滑膜肉瘤罕见,需要与肾脏其他一些梭形细胞肿瘤相鉴别,分子遗传学检测SYT-SSX融合mRNA有助于其诊断和鉴别诊断。     

Follicular dendritic cell sarcoma mimicking giant cell carcinoma of the pancreas

Extranodal follicular dendritic cell (FDC) tumors are rare. Recognition of the morphological spectrum of FDC tumors is important to clinical diagnosis. Herein is presented a case of pancreatic FDC sarcoma with unusual clinicopathological features. A 64-year-old male patient presented with weight loss, poor appetite, abdominal fullness, mild anemia and mild peripheral eosinophilia. Histologically, the tumor was composed of both epithelioid and spindle cells with abundant intracytoplasmic hyaline globules. These tumor cells were positive for CD21, CD23, CD35, S-100 protein, fascin and clusterin. Both epithelioid and spindle tumor cells independently colonized the liver and formed two tumor nodules 18 months after the initial resection. Notably, the two hepatic metastases additionally acquired patchy expression of human leukocyte antigen-DR. The epithelioid FDC in one of the hepatic lesions transformed into numerous bizarre giant cells, which could easily be confused with a metastatic giant cell carcinoma from the pancreas. FDC tumor should therefore be included in the differential diagnoses when dealing with a giant cell tumor.     

子宫内膜间质肉瘤55例临床病理特点和预后分析

目的 了解子宫内膜间质肉瘤(ESS)的病理形态特征并分析影响预后的相关指标.方法 收集该院55例ESS患者的临床和病理资料,所有病例重新阅片,参照文献分类为低级别子宫内膜间质肉瘤(LGESS)、不伴核多形的未分化子宫内膜肉瘤(UES-U)、伴有核多形的未分化子宫内膜肉瘤(UES-P);同时观察肿瘤细胞的形态特点,包括纤维样、肌样、黏液样、上皮样分化,并计数核分裂象等.对所有病例进行临床资料的收集并随访.结果 LGESS、UES-U、UES-P型病例分别为39、9、7例.病理形态上,ESS有多种形态分化并存的特点,LGESS、UES-U及UES-P型病例中分别有12.8%(5/39)、5/9及5/7伴有两种以上混合的形态学分化;同一病例的不同区域核分裂象计数和组织学类型亦存在较大差异.临床上肿瘤复发比例分别为51.6%(16/31)、5/6、2/3;LGESS无死亡病例,UES-U和UES-P中各有2例死亡,且UES-U的死亡病例均有局灶UES-P区域.按核分裂象最高计数进行预后分析,≥10/10 HPF的病例复发率显著高于＜10/10 HPF的复发率(P=0.009),在LGESS病例中亦存在这种统计学差异,所有死亡病例的核分裂象最高计数均＞30/10 HPF.结论 ESS常见不同程度分化重叠及多向分化的特点,尤以UES-U和UES-P中更为常见,因此应充分取材以寻找诊断线索.肿瘤中伴有UES-P图像,同时伴核分裂象计数高度活跃可能会增加死亡风险.在LGESS病例中,核分裂象最高计数≥10/10 HPF的肿瘤复发率显著增高,在诊断时应引起重视.

Abstract：

Objective To investigate the clinicopathologic features and the prognostic factors of endometrial stromal sarcoma (ESS). Methods 55 cases of endometrial stromal sarcoma were reviewed and categorized into 3 pathologic types based on the related literatures, i.e. , low grade endometrial stromal sarcoma (LGESS), undifferentiated endometrial sarcoma with nuclear uniformity (UES-U) and undifferentiated endometrial sarcoma with nuclear pleomorphism (UES-P). Meanwhile, the pathologic features were reviewed, including fibroid, myoid, mucoid, and epithelioid differentiation and mitotic index.Clinical and follow-up data were collected. Results In endometrial stromal sarcoma, two or three pathologic types co-existed in one case, including 12. 8% (5/39) of LGESS, 5/9 of UES-U, and 5/7 of UES-P.Mitotic index varied in different regions of one tumor from rare to high. Multi-differentiation was also commonly seen in ESS. The numbers of cases in LGESS, UES-U and UES-P were 39, 9 and 7, with recurrence rate of 51.6% ( 16/31 ), 5/6 and 2/3, respectively. There was no death case in LGESS, and 2 cases were died in UES-U and UES-P, respectively. In the 2 death cases of UES-U, both had focus of UES-P. There was a significant difference in the recurrence rate between cases with different mitotic index ( ≥ 10/10 HPF and ＜ 10/10 HPF, P = 0. 009), especially in LGESS group. All death cases had high mitotic index ( ＞ 30/10 HPF). Conclusions It is a common phenomenon in ESS that two or three pathologic types may exist in one case, especially in UES-U and UES-P. And multi-differentiation is also commonly seen in ESS. So adequate pathologic sampling is important for pathologists to make a correct diagnosis of ESS in daily work. The recurrence rates are significantly higher in cases with high mitotic index,especially in LGESS. In addition, the presence of UES-P and high mitotic index may increase the risk of death in the patients.