Epithelioid sarcoma. Report of a case with fine needle aspiration diagnosis

BACKGROUND: Epithelioid sarcoma is a rare type of soft tissue sarcoma affecting the extremities, particularly the hands and fingers. Though it is well described histopathologically, publications regarding its cytologic findings are limited. CASE: A 52-year-old woman presented with swelling of the left middle finger. Fine needle aspiration was performed. Smears showed oval to polygonal cells with epithelioid features. A diagnosis of soft tissue sarcoma with a possibility of epithelioid sarcoma was suggested. Histopathologic examination and immunohistochemistry confirmed the diagnosis. CONCLUSION: In the presence of classic cytologic findings, the diagnosis of epithelioid sarcoma can be suggested. Subsequent histologic examination and immunohistochemistry can confirm the diagnosis.     

Fine needle aspiration cytology of primary epithelioid sarcoma. A report of 2 cases

BACKGROUND: Epithelioid sarcoma is a rare soft part tumor, the cytologic features of which have not been fully elucidated to date. We describe the cytologic features in 2 cases of primary epithelioid sarcoma with samples obtained by fine needle aspiration (FNA). CASES: Case 1 was a 50-year-old male who complained of a small mass in his left palm. Case 2 was a 56-year-old female who presented with a mass on the medial aspect of her right forearm. Preoperative FNA smears in both cases showed loose, aggregated and isolated tumor cells that were round to polygonal, with eccentrically located nuclei, against a background of inflammation and necrosis. The tumor cells showed moderate atypia, irregularity in size and many mitoses. In case 1 a presumptive diagnosis of epithelioid sarcoma was made by FNA cytology, while in case 2, FNA cytology revealed a high grade sarcoma with abundant matrix mimicking osteoids, difficult to differentiate from an extraskeletal osteosarcoma. CONCLUSION: Epithelioid sarcoma may be difficult to differentiate from an extraskeletal osteosarcoma in cases with abundant hyalinized collagen on FNA cytology.     

Fine needle aspiration cytology of primary proximal-type epithelioid sarcoma of the perineum: a case report

BACKGROUND: Epithelioid sarcoma is a malignant soft tissue tumor of unknown histogenesis. We describe the cytologic findings in a case of primary proximal-type epithelioid sarcoma of the perineum and results of an immunofluorescence analysis of rhabdoid cells from this tumor. To the best of our knowledge, the 3-color immunofluorescence features of proximal-type epithelioid sarcoma have never before been reported. CASE: An 8-cm-diameter mass with a 2.5-cm ulcer was found in the perineum of a 36-year-old man. After excision of the tumor, histopathologic examination of the resected specimen suggested a diagnosis of proximal-type epithelioid sarcoma. Fine needle aspiration cytology showed numerous rhabdoid cells with globular intracytoplasmic inclusions. A few isolated cells and polygonal cells were also observed in the smears. Three-color immunofluorescence analysis indicated that the intracytoplasmic inclusions in the rhabdoid cells were positive for cytokeratin, vimentin and CD34. The cytoplasmic staining pattern differed between rhabdoid and epithelioid sarcoma cells. CONCLUSION: Immunofluorescent staining of rhabdoid cells from a primary perineal proximal-type epithelioid sarcoma revealed an uneven distribution of cytokeratin in intracytoplasmic inclusions, with the highest concentration at the periphery of the inclusions.     

Primary epithelioid sarcoma of the scalp.

This article retrospectively reviewed a case of epithelioid sarcoma of the scalp; a treatment plan for this type of neoplasm has not been well defined in the literature because of the rarity of sarcomas in general and sarcomas located in the head and neck in particular. No comparative results can be drawn when dealing with such lesion in the scalp. Early recognition with an aggressive approach to confirm the existence of an epithelioid sarcoma is mandatory; a high index of diagnostic awareness is needed to recognize this uncommon tumor. Early diagnosis can only be auspicious. Early wide surgical resection is imperative to ensure better control of imperceptible tumor extension, and well-planned diagnostic and therapeutic intervention, rather than isolated management and referral for adjunctive treatment, is important.     

Fine needle aspiration cytology of epithelioid angiosarcoma. Report of a case with nuclear grooves and indentations

BACKGROUND: Angiosarcoma, because of its rarity and histologic diversity, has been a persistent challenge to cytopathologists. Epithelioid angiosarcomas are often confused with carcinomas, melanomas and other epithelioid sarcomas, both cytologically and histologically. Here we report the cytopathologic features of a case of epithelioid angiosarcoma with prominent nuclear grooves. CASE: A 61-year-old male visited the hospital with a neck mass. On fine needle aspiration the hypercellular smear, with a lymphocytic bloody background, showed variable-sized, round to oval, pleomorphic cells with frequent nuclear grooves, indentations and plump cytoplasm. The cells were diffusely scattered or loosely aggregated, with occasional acinar and cell-in-cell configuration. The case was diagnosed as epithelioid angiosarcoma on excisional biopsy. CONCLUSION: Whenever fine needle aspiration cytology, especially in the head and neck area, suggests malignancy composed predominantly of epithelioid cells, epithelioid angiosarcoma should be considered. The differential diagnosis should include metastatic tumors, such as carcinoma, melanoma and primary sarcoma. Nuclear grooves and indentations are 1 of the important diagnostic features of epithelioid angiosarcoma.     

Epithelioid and fibroblastic rat kidney cell clones: epidermal growth factor (EGF) receptors and the effect of mouse sarcoma virus transformation.

Fibroblastic and epithelioid clones have been isolated from the normal rat kidney line, NRK. These clones were studied for their ability to bind epidermal growth factor (EGF), susceptibility to transformation by mouse sarcoma virus (MSV), and alteration in EGF binding upon sarcoma virus transformation. The epithelioid clones bound much more EGF than the fibroblastic clones; Scatchard plots on two of these clones, one epithelioid and one fibroblastic, showed that the higher EGF binding (1.3 x 10(5) molecules per cell for the epithelioid clone and 1.3 x 10(4) molecules per cell for the fibroblastic clone) was due to a greater number or receptors on the epithelioid cells rather than to a difference in the apparent affinity constant. When the clones were transformed by Moloney murine sarcoma virus the EGF binding decreased, the effect being greater with the fibroblastic clones. In 20 out of 20 independently isolated sarcoma virus transformed fibroblastic clones, the level of EGF binding was either greatly reduced or completely eliminated. In contrast to EGF, another growth factor, multiplication stimulating activity (MSA), bound to a greater extent to the fibroblastic clones than the epithelioid clones, and its binding was not decreased by sarcoma virus transformation. The results show that loss of EGF binding ability correlates with expression of the murine sarcoma virus transformation.     

Epithelioid sarcoma

The first appearance, the 12-year follow-up, and the autopsy of a patient with a strange soft-tissue tumor in the lower leg region are described. Although it was originally taken for a malignant synovial tumor, its peculiar distribution pattern, together with its histologic picture, confirmed the final diagnosis as an epithelioid sarcoma.     

Genetics of rare mesenchymal tumors: Implications for targeted treatment in DFSP,ASPS, CCS,GCTB and PEComa

Soft tissue and bone sarcomas comprise a heterogeneous group of mesenchymal tumors that include roughly 130 distinct diagnostic entities. Many of them are exceptionally rare, with only few cases diagnosed worldwide each year. Development of novel targeted treatment in this group of tumors is of special importance since many sarcoma subtypes are resistant to conventional chemotherapy and the effective therapeutic options are limited. In this review we aim to discuss the molecular implications for targeted therapy in selected rare soft tissue and bone sarcoma subtypes, including dermatofibrosarcoma protuberans (DFSP), alveolar soft part sarcoma (ASPS), clear cell sarcoma (CCS), giant cell tumor of bone (GCTB) and perivascular epithelioid cell neoplasms (PEComas).This article is part of a Directed Issue entitled: Rare cancers.     

Solitary fibrous tumor of the thigh with epithelioid features: a case report

Background

Extrapleural Solitary Fibrous tumors (SFTs) have been increasingly reported. The retroperitoneum, deep soft tissues of proximal extremities, abdominal cavity, trunk, head and neck are the most common extraserosal locations reported. Microscopically they show a wide range of morphological features, and so the differential diagnosis is extensive. Immunohistochemically, they commonly express CD34, vimentin, bcl-2 and CD99. Epithelial membrane antigen (EMA) and smooth muscle actin (SMA) may occasionally be expressed. Epithelioid morphology in extrapleural SFT has only very occasionally been described (five cases reported), some of them with biphasic pattern and others with malignant characteristics.

Case presentation

A SFT of the thigh with epithelioid areas in a 63 year old woman is reported. Microscopically the tumor showed areas hypo and hipercellular. At the periphery of the hipercellular areas there were nodules composed of epithelioid cells. Immunohistochemically both the spindle and epithelioid cells were positive for CD34, vimentin, bcl-2 and CD99. Epithelial, neural and muscular markers were negative. Molecular study was done and ruled out a synovial sarcoma.

Conclusion

Ten cases of SFT of the thigh have been reported but to our knowledge this is the first case with epithelioid morphology affecting the extremities. Identification of this pattern of SFT is of importance, to avoid misdiagnosis with other more aggressive conditions in soft tissue.

     

Acral myxoinflammatory fibroblastic sarcoma in FNAB samples: can we distinguish it from other myxoid lesions?

We analysed cytomorphological characteristics of three fine needle aspiration biopsy (FNAB) samples of acral myxoinflammatory fibroblastic sarcoma (AMIFS) as well as the features of a number of other benign and malignant myxoid lesions. The analysis showed that FNAB samples from two cases of AMIFS had similar cytomorphology, containing all the characteristic features described in surgical biopsies: myxoid material, spindle cells with bipolar cytoplasmic extensions, epithelioid cells with globules of extra-cellular material, ganglion-like and lipoblast-like giant cells. Only the inflammatory component was scarce. The third sample did not contain features typical of AMIFS. Samples from other myxoid tumours resembled AMIFS to some extent, however, none of them contained all three tumour components characteristic of AMIFS. Cytomorphology of AMIFS may be characteristic enough to enable a definitive diagnosis from FNAB, provided all the distinctive features are sampled.     

Critical appraisal of fine needle aspiration cytology in tuberculous lymphadenitis.

A cytomorphologic diagnosis of tuberculous lymphadenitis by examination of needle aspirates was made in 560 of 1,471 cases of lymphadenopathy studied over two years. Cytologic features were categorized into four groups: epithelioid clusters with or without Langhans's giant cells without necrosis (32.14%), epithelioid clusters with or without Langhans's giant cells with necrosis (50.35%), occasional epithelioid cells without characteristic necrosis/giant cells (2.85%) and necrosis without epithelioid clusters or Langhans's giant cells (14.64%). While a diagnosis of tuberculous lymphadenitis was offered with confidence in the first two groups, constituting about 82.49% cases, aspirates from the third- and fourth-group patients were subjected to Ziehl-Neelsen staining for acid-fast bacilli, which was positive in 12.5% and 75.6% of cases, respectively.     

Fine needle aspiration cytology of suspected tuberculous lymphadenitis

Fine needle aspiration cytology of suspected tuberculous lymphadenitis The aims of this cross-sectional study were to describe the distributional patterns of tuberculous lymphadenitis and to assess the correlation between fine needle aspiration cytology (FNAC) and the Ziehl Neelsen staining technique in diagnosing tuberculous lymphadenitis. Romanowsky's method (Wright's stain) for cytological diagnosis and Ziehl Neelsen (hot method) for the identification of acid-fast bacilli were utilized. Out of one hundred and twenty-eight consecutive patients attending the cytological diagnostic service of the Department of Pathology within Jimma University, 89 (69.6%) of the patients were younger than 30 years of age. The male to female ratio was 1.3 : 1. The cervical region was the most common site and involved 95 cases (74.2%), followed by the axillary and inguinal lymph node regions (20.3% and 4.3%, respectively). The Wright's-stained cytology smears were grouped into three categories: epithelioid granulomas without necrosis, epithelioid granulomas with caseous necrosis and necrosis without epithelioid granulomas. The Ziehl Neelsen stains were undertaken on separate slides: 20.0% of the cases showing epithelioid granulomas without necrosis, 61.9% of those with epithelioid granulomas with necrosis/abscesses and 69.7% of those with necrosis without granulomas were found to be positive for acid-fast bacilli. The overall positivity for the ZiehlNeelsen stained cases was 59.4%. It can therefore be concluded that FNAC is a reliable diagnostic tool in helping to avert the more invasive surgical procedures undertaken in the diagnosis of tuberculous adenitis. The ZiehlNeelsen stain for identification of acid-fast bacilli should be incorporated as an adjunct to increase the diagnostic accuracy of tuberculous lymphadenitis.     

Fine needle aspiration of toxoplasmic (Piringer-Kuchinka) lymphadenitis: a cytohistologic correlation study

OBJECTIVE: To define the cytomorphologic features of toxoplasmic lymphadenitis (TL) and to establish the diagnostic sensitivity and specificity of the cytologic diagnosis. STUDY DESIGN: A cytohistologic correlation study of 11 patients in which a histologic diagnosis of TL was available. These cases were analyzed in a blind study among other cases of nonneoplastic lymphadenopathies. The results of the study are expressed in terms of diagnostic sensitivity and specificity. Although not included in the study, 3 other cases of TL with serologic confirmation were also reviewed. RESULTS: Microgranulomas were a characteristic finding in TL. They consisted of small clusters of epithelioid histiocytes, each with abundant cytoplasm and an eccentric, oval nucleus. Diff-Quik-stained smears had cytoplasm with particularly pale staining and a homogeneous appearance. Most microgranulomas had a monotonous appearance with a few, small lymphocytes accompanying the epithelioid cells. No necrosis, suppurative changes or giant cells were present. Blind examination by 4 reviewers led to a correct diagnosis of TL in 9 of the 11 cases. Eight of the 9 cases were recognized by each of the 4 reviewers. One false positive diagnosis was made by 1 of the reviewers. The sensitivity of the diagnosis was 72.7-81.8% and the specificity 98.8-100%. CONCLUSION: This study showed high sensitivity and specificity for the cytologic diagnosis of TL. Given the appropriate clinical context, the presence of characteristic epithelioid microgranulomas permits a diagnosis of TL. The cytologic diagnosis can be easily confirmed with serologic studies, thereby avoiding biopsy.     

甲状腺上皮样血管内皮瘤临床病理及分子生物学特点分析

为探讨甲状腺上皮样血管内皮瘤的病理形态特征、免疫表型和鉴别诊断,对2例甲状腺上皮样血管内皮瘤进行临床资料分析、随访,并观察其病理形态及免疫组织化学染色分析。2例甲状腺上皮样血管内皮瘤均位于甲状腺下极,实性,切面暗红色,镜下由增生的血管内皮细胞构成,具有上皮样细胞形态,胞浆较丰富,嗜酸性,多呈空泡状,细胞间可见血管腔,免疫组化:CD34(+)、Vim(+)、细胞角蛋白(cytokeratin,CK)(-)、甲状腺球蛋白(thyroid globulin,TG)(-)。上皮样血管内皮瘤是一种罕见的甲状腺肿瘤,其生物学行为属于低度恶性。     

Caseous, necrotic material and epithelioid cell granulomas in synovial fluid from a patient with tuberculous infection: a case report

BACKGROUND: The gross appearance and cytologic findings in synovial fluid in tuberculous infections are similar to those in other types of chronic synovial effusion. Demonstration of acid-fast bacilli (AFB) is required for a definitive diagnosis of tuberculous effusion; it is reported in only 20% of cases. The presence of frank caseous necrotic material and epithelioid cell granulomas in synovial fluid samples is unusual but strongly indicative of tuberculous infection. CASE: A 28-year-old man, on treatment for pulmonary tuberculosis, presented with a history of right ankle swelling, which was clinically interpreted as nonspecific synovitis. The synovial fluid was yellowish, with fluffy, whitish material. Cytologic smears showed abundant, caseous, necrotic material; a few histiocytic aggregates; and occasional epithelioid cell granulomas. Although stain for AFB was negative, considering the clinical presentation, a diagnosis of tuberculous synovitis could be rendered. CONCLUSION: Caseous, necrotic material and epithelioid granulomas in synovial fluid are highly unusual but, when present, can be considered definitive evidence of tuberculous effusion, particularly in a known case of pulmonary tuberculosis.     

Lymph node metastasis of soft tissue tumors: a cytomorphologic study

OBJECTIVE: To study the frequency of regional lymph node metastasis of soft tissue tumors (STT) and to evaluate the utility of fine needle aspiration cytology (FNAC) as an initial investigative modality. STUDY DESIGN: A prospective and retrospective study of over 6 years (1998-2004) was performed to look for frequency of STT metastasizing to lymph nodes. FNAC of enlarged nodes was performed as a routine outpatient procedure after obtaining complete clinical details. Histopathology and immunohistochemistry were correlated where available. RESULTS: Lymph node enlargement was seen in 23 of 241 patients with STTs, of which 19 cases showed involvement (7.88%), synchronous with primary in 12 cases and metachronous in 7 cases. The most common sites of primary tumor were the lower extremity and head and neck region with involved regional lymph nodes. STTs commonly involving lymph nodes were rhabdomyosarcoma and extraskeletal Ewing's/primitive neuroectodermal tumor (PNET); other rare tumors included malignant granular cell tumor, epithelioid hemangioendothelioma, mediastinal ganglioneuroblastoma, angiosarcoma and epithelioid sarcoma. CONCLUSION: Lymph node aspirates should be examined for alien cells, particularly smears that are paucicellular and demonstrate cystic change. Lymph node metastasis of STT is rare and influences staging, treatment and prognosis. Enlarged regional nodes should be examined with FNAC.     

Pathology of cat-scratch disease

A pathologic and histogenetic study of material obtained from ten cases of cat-scratch disease was carried out. The earliest lesion was of ten days' duration and the oldest of 35 days' duration. The first changes in lymph nodes consisted of proliferation of epithelioid cells followed by exudation of leukocytes in their centers and subsequent necrosis of the exudate and epithelioid cells. Proliferative changes leading to formation of epithelioid cell tubercles were seen in some cases. Homogenization of necrotic centers brought about the formation of caseous tubercles similar to those seen in tuberculosis, syphilis, lymphogranuloma venereum and tularemia. Differentiation and the final diagnosis of cat-scratch disease rests upon correlation of histopathologic observations, clinical studies and specific skin tests.     

Use of fine needle aspiration cytology in leprotic lesions: a report of 4 cases

BACKGROUND: Although a few studies have shown fine needle aspiration cytology (FNAC) to be a sensitive diagnostic tool in the detection of nerve involvement, its role as an initial diagnostic procedure in pure neuritic leprosy (PNL) and in the detection of skeletal lesions with unusual findings has not been documented before. CASES: Three patients who presented with thickened nerves and a fourth with biopsy-proven lepromatous leprosy with lesions in hand bones underwent FNAC. Of the 3 patients with nerve thickening, 2 had a clinical suspicion or diagnosis of neuritic leprosy, whereas in the third patient a clinical differential diagnosis of a soft tissue tumor or parasitic cyst was considered. FNAC in all 3 cases revealed epithelioid cell granulomas, Langhans giant cells and caseous necrosis. Fites and Ziehl-Neelsen stains were negative for acid-fast bacilli. Cytologic diagnosis of pure neuritic leprosy was made in all 3 cases and confirmed by histopathologic examination. FNAC of skeletal lesions from the fourth patient confirmed involvement of bone with unusual cytologic findings of epithelioid cell granulomas and giant cells along with a significant proportion of foamy macrophages and strong Fites stain positivity. CONCLUSION: FNAC is a simple, useful, minimally traumatic and routinely applicable procedure in the diagnosis of pure neuritic leprosy and leprous osteitis.     

Fine needle aspiration cytology of localized Leishmania lymphadenitis

Leishmania lymphadenitis, an uncommon cause of lymphadenopathy, is usually diagnosed by surgical biopsy performed because of suspicion of lymphoma. The cytopathologic features of this disease do not appear to have been previously described. This paper describes the findings in seven cases of Leishmania lymphadenitis diagnosed by fine needle aspiration (FNA) cytology. The identification of Leishman-Donovan bodies in epithelioid cells in the aspirates led to the cytologic diagnosis of Leishmania lymphadenitis, which was histologically confirmed in all cases. Since this disease is self-limiting and requires no treatment, FNA diagnosis is especially useful in that the patient can be spared more invasive diagnostic techniques.     

Comparative analysis of electron microscopy and immunocytochemistry in the cytologic diagnosis of malignant small round cell tumors

OBJECTIVE: To compare the contributions of electron microscopy (EM) and immunocytochemistry (ICC) as adjuncts in the cytodiagnosis of malignant small round cell tumors (MSRCT). STUDY DESIGN: This prospective study included 57 cases with a preliminary aspiration diagnosis of MSRCT. The contributions of EM and ICC in arriving at a specific diagnosis were evaluated. RESULTS: The 57 cases included 22 cases of Ewing's sarcoma/peripheral neuroectodermal tumor (PNET), 12 neuroblastomas, 8 Wilms' tumors, 6 rhabdomyosarcomas, 5 lymphomas, 2 retinoblastomas and 1 synovial sarcoma. One case remained unclassified. Electron microscopy was crucial to the diagnosis in 38.4% cases as against 39.2% of cases by ICC. The light microscopic diagnosis was confirmed in 42.3% and 53.5% cases by EM and ICC, respectively. EM and ICC were inconclusive for a specific diagnosis in 19.2% and 7.1% of cases, respectively. Technically unsatisfactory preparations in EM and ICC accounted for 5 and 1 cases, respectively. The overall efficiency in making a diagnosis was 80.7% for EM versus 92.8% for ICC. Aberrant expression of antigens led to difficulties in interpretation of ICC, and EM was particularly helpful. The ultrastructural demonstration of neural differentiation in Ewing's sarcoma/PNET tumors helped place tumors in the PNET category. CONCLUSION: While ICC is the ancillary method of choice in the cytologic diagnosis of MSRCT, EM contributes to the diagnosis and improves diagnostic accuracy.