Fine-needle aspiration cytology of alveolar soft-part sarcoma

Alveolar soft-part sarcoma (ASPS) is a rare soft-tissue tumor. Few cases have been reported in the aspiration cytology literature. We discuss the fine-needle aspiration (FNA) cytologic features of ASPS in four patients. The smears were characterized by single cells and clusters of cells associated with thin-walled vasculature, resulting in a distinct pseudoalveolar pattern. The cells had abundant granular cytoplasm and large round nuclei with prominent central nucleoli. Binucleated and occasional multinucleated cells were present. Numerous stripped tumor nuclei were a consistent feature. Periodic acid-Schiff (PAS)-positive diastase-resistant granules were demonstrable within the intact and fragmented cytoplasm. Electron microscopy of aspirated material identified granules and crystals with the characteristic lattice pattern. To conclude, the FNA cytologic features of ASPS are characteristic. Confirmation by special stains and electron microscopy of FNA material is possible. The differential diagnostic considerations include renal cell carcinoma, paraganglioma, granular cell tumor, clear cell sarcoma, and epithelioid sarcoma.     

Malignant Round Cell Tumor of Bone with Neural Differentiation (Neuroectodermal Tumor)

A malignant round cell tumor primary in bone is reported in a young girl, showing histologically rosette formation and pseudoalveolar spaces. Vimen-tin, NSE, and S-100 were positive. Electron microscopy demonstrated abundant glycogen and neural features such as cell processes, microtubules, and occasional dense-core granules. Whether this neuroectodermal tumor represents a separate entity or is a variant of Ewing's sarcoma should wait for further experience, in the light of recent evidence suggesting that Ewing's sarcoma arises from primitive multipotential cells.     

Malignant Round Cell Tumor of Bone with Neural Differentiation (Neuroectodermal Tumor)

A malignant round cell tumor primary in bone is reported in a young girl, showing histologically rosette formation and pseudoalveolar spaces. Vimen-tin, NSE, and S-100 were positive. Electron microscopy demonstrated abundant glycogen and neural features such as cell processes, microtubules, and occasional dense-core granules. Whether this neuroectodermal tumor represents a separate entity or is a variant of Ewing's sarcoma should wait for further experience, in the light of recent evidence suggesting that Ewing's sarcoma arises from primitive multipotential cells.     

Fine-needle aspiration cytology of sarcoma: Retrospective review of diagnostic utility and specificity

Fine-needle aspiration cytology (FNA) is useful because of its low cost, quick turn around time, and low incidence of complications. This study investigates the role FNA plays in the evaluation of sarcoma. We reviewed all the pathologic material from patients with sarcoma or a FNA diagnosis suggestive of sarcoma at the University of California-Davis Medical Center (1985–1994). Fifty-two of 196 patients identified (26.5%) were evaluated with FNA exhibiting 46 soft tissue and 6 bone lesions located in the extremities (19), trunk (19), head and neck (8), retroperitoneum (3), and abdominal cavity (3). Among 196 patients identified, those evaluated by FNA had soft tissue rather than bone lesions (P < .001) and primary sites other than in the extremities (P < .001). The primary neoplasms for the 52 FNA patients included 47 sarcomas (10 malignant fibrous histiocytomas and 16 other types), I intramuscular myxoma, 2 lymphomas, and 2 spindle cell (sarcomatoid) carcinomas. In 43 patients (22% of total), FNA was used first, before a primary diagnosis. The FNA report showed the correct specific neoplasm in only 20.9%. However, the FNA reports reflected an effective positive diagnosis leading to appropriate diagnostic biopsy in 88.4%, while only 7.0% were misleading (benign or inflammatory) and 4.6% inadequate. Review of FNA slides for 32 of 43 patients in which FNA was used first, in a blinded fashion, exhibited 21.9% specificity for the specific neoplasm, and 88.4% effective positive diagnoses. In 9 patients, FNA was utilized to investigate recurrence. Five out of 5 instances of recurrent sarcoma were identified by FNA. However, FNA misidentified recurrence in 3/4 instances, exhibiting suspicious cells from regenerating skeletal muscle (2) and a traumatic neuroma (I). The specific diagnosis for sarcomas is challenging even with surgical material. FNA usually does not provide a specific diagnosis (only in 20.9% of cases) and review of routinely prepared slides showed the same specificity as reflected by the original FNA report, at times formulated with the benefit of immunohistochemistry or electron microscopy. FNA effectively evaluated lesions in 45 of 52 patients (86.5%), confirming the useful screening role for this technique in sarcomas. Diagn Cytopathol 1996;15:23–32. © 1996 Wiley-Liss, Inc.     

软组织肿瘤细胞中的微丝结构

用透射电镜观察了一组18例肿瘤细胞胞浆中有微丝结构的软组织恶性肿瘤,电镜观察结果与光镜诊断不同,主要发生在纤维肉瘤、平滑肌肉瘤和恶性纤维组织细胞瘤中。肿瘤细胞膜下的具有密体的带状微丝可见于上述各种肿瘤中。一些肿瘤细胞也具有特征性的微丝结构。说明上述肿瘤的细胞成分有重叠。提出肿瘤的分类应主要依据大多数肿瘤细胞的分化方向来确定而不是臆测其组织发生和细胞来源。按此方法分类将会出现更多的纤维组织细胞瘤、肌纤维母细胞瘤和肌纤维组织细胞瘤的诊断。     

Fine-needle aspiration cytology of "proximal-type" epithelioid sarcoma

The cytologic and immunocytologic findings in a case of recurrent "proximal-type" epithelioid sarcoma (ES) of the vulva are presented. This is a recently described neoplasm that differs clinically and morphologically from conventional ES. Cytologic smears showed a dissociated population of large, atypical neoplastic cells with bi- and multinucleated cells, abundant cytoplasm, and rhabdoid-like morphology. Due to its different clinical management it must be differentiated from metastatic carcinoma and melanoma. From a practical perspective, its differentiation from other epithelial-like sarcomas is less important. In conclusion the cytopathologic findings of "proximal-type" ES show a good correlation with histopathology, permitting the diagnosis of recurrences and metastases. When accompanied by adequate clinical information and ancillary studies, a specific preoperative recognition seems possible.     

Stem cell transcription factor SOX2 in synovial sarcoma and other soft tissue tumors

Background

SOX2 has gained considerable interest as a pluripotency inducing gene. Co-transfection of SOX2 together with NANOG, KLF4 and c-MYC into adult fibroblasts was able to generate pluripotent stem cells. SOX2 has been reported to be expressed in synovial sarcoma, a tumor being characterized by the SS18-SSX gene fusion forming part of the SWI/SNF chromatin remodeling complex that affects histone methylation. The role of SOX2 in this tumor type as well as other soft tissue tumor entities however is still poorly characterized. We analyzed SOX2 protein expression in soft tissue tumors. Alongside we tested Histone H3 expression (H3K27me3) in SOX2 positive cases to investigate this epigenetic mark and its correlation with the SOX2 status and clinicopathological parameters.

L‐type amino acid transporter‐1 and CD98 expression in bone and soft tissue tumors

L‐type amino acid transporter‐1 (LAT1) is expressed in many cancers. We examined LAT1 and CD98 expression immunohistochemically in surgically resected specimens of various bone and soft tissue tumors. Out of 226 cases, 79 (35%) were LAT1⁺ and 95 (42%) were CD98⁺. In bone tumors, LAT1 was highly expressed in osteoblastoma (89%), chondrosarcoma (50%), and osteosarcoma (60%); in soft tissue tumors, LAT1 was highly expressed in rhabdomyosarcoma (80%), synovial sarcoma (63%), Ewing's sarcoma (60%), epithelioid sarcoma (100%) and angiosarcoma (100%). In malignant soft tissue tumors, LAT1 expression was associated with higher histological grade. High CD98 expression was seen in many bone tumors of intermediate and high malignancy. Among soft tissue tumors, CD98 was expressed in tendon sheath giant cell tumor and malignant peripheral nerve sheath tumor (57%), Ewing's sarcoma (50%) and undifferentiated sarcoma (64%). Some of the malignant soft tissue tumors expressed both LAT1 and CD98. This study showed that LAT1 and CD98 was expressed in many malignant and intermediate bone tumors, and some malignant soft tissue tumors.     

Kaposi's sarcoma and malignant lymphoma in AIDS

Summary A 48-year-old homosexual with contacts in different countries, including Haiti, presented with multiple pigmented or bluish nodules on both lower legs and upper arms. He had a history of secondary syphilis, hepatitis B and herpes zoster ophthalmicus. Biopsies of the skin tumors revealed a typical Kaposi's sarcoma of low grade malignancy. The endothelial origin of the tumor was indicated by the presence of specific endothelial organelles (Weibel-Palade bodies) in the cytoplasma of the tumor cells. Erythrocytophagocytosis was found in tumor cells within and without the vascular channels. Laboratory tests were compatible with the clinical diagnosis of an acquired immune deficiency syndrome (AIDS) with a helper: suppressor T-lymphocyte ratio of 0.28 and a cutaneous anergy. In the course of the illness tumors of the stomach and duodenum were detected. Histology showed a malignant non-Hodgkin lymphoma of high grade malignancy. Within weeks the patient died in a cachectic state. Autopsy revealed a Kaposi's sarcoma of the skin with metastases in the stomach and a wide-spread malignant lymphoma in the gastrointestinal tract, in several visceral organs and in many lymph nodes.     

骨外Ewing肉瘤/外周原始神经外胚叶肿瘤的临床病理分析

目的探讨骨外Ewing肉瘤／外周原始神经外胚叶肿瘤的临床病理特征及诊断、鉴别诊断依据。方法18例骨外Ewing肉瘤／外周原始神经外胚叶肿瘤行常规病理检查及免疫组化染色,其中2例进行电镜检查。结果光镜显示肿瘤组织主要由小圆形或卵圆形细胞组成,免疫组化染色显示肿瘤细胞膜CD99强阳性,电镜显示肿瘤细胞质内有神经内分泌颗粒。结论骨外Ewing肉瘤/外周原始神经外胚叶肿瘤的诊断依赖病理特征,并需要与其他小细胞恶性肿瘤进行鉴别。     

Prophylaxis of tumors induced by carcinogens in inbred mice

Antitumor vaccines were obtained with the aid of a filtrate of the culture fluid after growth of a culture ofBacillus mesentericus AB-56 from the cells of a sarcoma K-239 induced by methylcholanthrene and of an undifferentiated sarcoma Rt induced by an extract of the plantRubia tinctorum. Immunization of inbred CBA mice and CBAxC57BL/j hybrids with the vaccines led to resistance to subsequent inoculation with the same tumors in 84–100% of cases. The effectiveness of vaccination depended on the time elapsing since the vaccine was prepared and on the quantity of vaccine material taken for testing postvaccinal immunity.Academician D. K. Zabolotnyi Institute of Microbiology and Virology, Academy of Sciences of the Ukrainian SSR, Kiev. (Presented by Academician of the Academy of Medical Sciences of the USSR, L. V. Gromashevskii.) Translated from Byulleten' Eksperimental'noi Biologii i Meditsiny, Vol. 78, No. 9, pp. 90–92, September, 1974.     

Diagnostic value of intermediate filament antibodies in clinical cytology

Summary Antibodies to intermediate filament (IF) proteins can distinguish the major tumor groups as shown by results with sectioned human material. In this study we evaluate the use of similar methods in the cytology of human tumors. Smears obtained from fine needle aspiration biopsies were investigated using well characterized antibodies, each specific for only one of the five types of intermediate filaments. Tumor cells of different carcinomas, thymomas, and the epithelial part of pulmonary blastomas were positive with antibodies recognizing cytokeratins. Tumor cells in non-muscle sarcomas, including lymphoma and Ewing's sarcoma, could be specifically identified with antibodies to vimentin. Tumor cells of muscle sarcomas were desmin-positive. Finally, tumor cells in pheochromocytoma and bronchus carcinoid were positive with antibodies specific for neurofilaments. Specimens were also examined in parallel using conventional cytochemical stains, such as May-Grünwald-Giemsa. In addition, in most cases sections of the tumor were examined both by histology and IF typing of frozen sections to confirm the diagnosis made on the cytologic specimens. The results show that IF typing is a valuable diagnostic aid in clinical cytology.     

Cytologic features of alveolar soft part sarcoma: report of three cases

Alveolar soft part sarcoma (ASPS) is a rare, high-grade, epithelial-like sarcoma that shows characteristic histopathologic findings. Although a chromosomal anomaly that seems specific has been recently described, its diagnosis is based on histologic and ultrastructural features. The tumor shows no specific immunohistologic findings. Cytologic features of three cases of ASPS are presented. Preoperative fine-needle aspiration (FNA) of the primary soft tissue tumor was performed in two cases. In another two, mediastinal and pulmonary and subcutaneous metastatic lesions were aspirated. In all cases the cytologic image was identical with numerous, dissociated, large neoplastic cells with round-to-plasmocytoid morphology. Cytoplasmic fragility and granularity with abundant, atypical, naked nuclei were present. In one case, FNA material was available for ultrastructural studies. It disclosed the characteristic cytoplasmic crystalline structures. A specific cytologic diagnosis of ASPS was given in all cases. In conclusion, ASPS is a rare neoplastic entity that shows a characteristic cytologic image. When accompanied by an adequate clinical context it permits specific preoperative recognition. While immunocytologic studies are helpful to exclude other neoplasms, ultrastructure may result in an exact diagnosis.     

Pleomorphic hyalinizing angiectatic tumor of soft parts: ultrastructural analysis of a case with original features

Pleomorphic hyalinizing angiectatic tumor, a rare neoplasm of uncertain lineage resembling malignant fibrous histiocytoma and schwannoma, was first described in 1996 by M. E. F. Smith et al. (Am Surg Pathol. 20:21-29). To date, less than 100 cases have been reported in the international literature. It occurs in subcutaneous and intramuscular soft tissues of extremities or trunk in adults without sex predilection. All lesions are composed of sheets and fascicles of spindled and pleomorphic cells associated with clusters of thick-walled ectatic vessels surrounded by a perivascular hyaline material and inflammatory cells such as mast cells. About one-half of these neoplasms express CD34. No patient has developed metastases but occasional local recurrences are possible. This tumor of uncertain lineage is suggested to be an aggressive locally growing low-grade sarcoma. Only 3 cases were previously studied by electron microscopy and appeared to consist of primitive fibroblastic cells. The authors report histological and ultrastructural characteristics of a new case of PHAT excised from the right buttock of a 66-year-old man with the presence of ganglion-like cells, a feature that has not been previously reported, and unusual central ischemic necrosis. The features of this case are suggestive of a fibroblastic origin.     

Role of electron microscopy in the evaluation of soft tissue neoplasms, with emphasis on spindle cell and pleomorphic tumors

The current significant role of transmission electron microscopy in the evaluation of soft tissue tumors when correlated with conventional histological and immunohistochemical studies is discussed for the following entities: myxofibrosarcoma, storiform-pleomorphic fibrosarcoma (malignant fibrous histiocytoma), and myofibrosarcoma; dermatofibrosarcoma protuberans; hemangiopericytoma; monophasic synovial sarcoma; extrarenal rhabdoid tumor; soft tissue perineurioma; and gastrointestinal stromal tumors, notably the so-called autonomic nerve variant.     

Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts: Ultrastructural Analysis of a Case with Original Features

Pleomorphic hyalinizing angiectatic tumor, a rare neoplasm of uncertain lineage resembling malignant fibrous histiocytoma and schwannoma, was first described in 1996 by M. E. F. Smith et al. (Am Surg Pathol. 20:21–29). To date, less than 100 cases have been reported in the international literature. It occurs in subcutaneous and intramuscular soft tissues of extremities or trunk in adults without sex predilection. All lesions are composed of sheets and fascicles of spindled and pleomorphic cells associated with clusters of thick-walled ectatic vessels surrounded by a perivascular hyaline material and inflammatory cells such as mast cells. About one-half of these neoplasms express CD34. No patient has developed metastases but occasional local recurrences are possible. This tumor of uncertain lineage is suggested to be an aggressive locally growing low-grade sarcoma. Only 3 cases were previously studied by electron microscopy and appeared to consist of primitive fibroblastic cells. The authors report histological and ultrastructural characteristics of a new case of PHAT excised from the right buttock of a 66-year-old man with the presence of ganglion-like cells, a feature that has not been previously reported, and unusual central ischemic necrosis. The features of this case are suggestive of a fibroblastic origin.     

Cytopathology of undifferentiated (embryonal) sarcoma of the liver

We have studied one undifferentiated (embryonal) sarcoma of the liver (USL) by fine-needle aspiration cytology (FNAC) and have correlated the cytologic findings with those seen in the histologic sections. The main cytologic and immunocytochemical features were: cellular aspirates, mesenchymal clusters, disparity in individual cell size (small round cells, and multinucleated giant cells, sometimes bizarre), absence of biliary pigment, AFP-negative and vimentin-negative tumor cells, AFP-negative and PAS-positive hyaline globules, and apoptotic cells. Only immunostaining for alpha-1-antitrypsin, alpha-1-anti-chymotrypsin, and carcinoembryonic antigen were positive in tumor cells in histologic sections. The differential diagnosis should be carried out with rhabdomyosarcomas, hepatoblastomas, malignant fibrous histiocytomas, and poorly differentiated hepatocarcinomas. We think that the cytologic features observed in this case, evaluated both in the appropriate clinical context and in the light of the laboratory findings, may permit the correct diagnosis of this infrequent liver tumor. Nevertheless, more cases need to be studied to assess the reliability of our findings. © 1995 Wiley-Liss, Inc.     

Endolymphatic Stromal Myosis with Focal Tubular-Glandular Differentiation (Biphasic Endometrial Stromal Sarcoma)

A case of low grade endometrial stromal sarcoma with tubular-glandular differentiation is reported. The tumor showed a characteristic pattern of clusters and trabeculae of spindle-shaped cells infiltrating the myometrium. Lymphatic invasion was evident. Foci of solid, alveolar pattern and cleft tubular spaces were seen. The tubular spaces were lined by a cuboidal epithelium, which at the ultrastructural level showed blunt microvilli, and between adjacent cells junctional complexes and desmosomes. Both the spindle-shaped cells and the epithelial cells were rich in cytoplasmic filaments. The latter did not react with serum containing antibody against actin using a two-layer immuno-peroxidase method. The ultrastructural resemblance between the epithelial and spindle-shaped cells favors a common cellular origin, the endometrial stromal fibroblast.     

Spectrum of cytomorphological features, including literature review, of an extraskeletal myxoid chondrosarcoma with t(9;22)(q22;q12) (TEC/EWS) results in one case

Extraskeletal myxoid chondrosarcoma (EMC) is an uncommon soft tissue sarcoma with evolving literature on its cytomorphological features and limited documentation of its molecular analysis. Herein, we present cytological features, including review, of four cases of an EMC. Smears were predominantly hypercellular, comprising tumor cells arranged in clusters, traberculae, and cords against a variable chondromyxoid background. Cells were mainly polygonal shaped with round to indented nuclei, uniform chromatin, displaying intranuclear inclusions, grooves, and eosinophilic to finely vacuolated cytoplasm. Three cases revealed presence of "rhabdoid" cells. All cases had histopathologic confirmation. One case displayed t(9;22)(q22;q12) translocation by fluorescent in situ hybridization (FISH), on smears.