Histogenesis of alveolar soft part sarcoma. An immunohistochemical and biochemical study

In order to clarify the histogenesis of alveolar soft part sarcoma (ASPS), an immunohistochemical and biochemical study was performed on three cases. The immunohistochemical study indicated the presence of actin, desmin, vimentin, and Z-protein in all cases. On the other hand, intermediate filaments other than desmin and vimentin were not detected immunohistochemically. The presence of desmin and Z-protein strongly suggests the myogenic character of this tumor. As to whether ASPS shows striated muscle differentiation or smooth muscle differentiation, the immunohistochemical absence of myoglobin in the three cases suggests that the tumor does not differentiate in the direction of striated muscle. However, biochemical assay of subunits of enolase revealed significantly high amounts of beta-enolase, which is known as a marker for striated muscle, in all three cases. The determined values--735, 426, and 584 ng/mg of protein --are indicative of striated muscle differentiation. In addition, the immunohistochemical study of all cases revealed the presence of beta-enolase in tumor cells. These data definitely show the myogenic character and rhabdomyoblastic differentiation of ASPS.     

Penile epithelioid sarcoma

Emphysematous pyelonephritis (EPN) is an uncommon and potentially life-threatening necrotizing inflammation of the renal parenchyma. EPN associated with autosomal dominant polycystic kidney disease (ADPCK) is extremely rare. We report such a case of bilateral EPN with ADPCK that was successfully treated with conservative methods. To our knowledge, our case is only the second to document bilateral EPN occurring with ADPCK and the first one to be treated successfully with conservative methods.     

Chest wall epithelioid sarcoma

Two relatively rare cases of epithelioid sarcoma located in the chest wall are reported herein. The first was a tumor, 3.8 x 5.5 x 2.4 cm in size, originating in the left erector spinae muscle of a man 64 years of age. He experienced local recurrence 3 times even after wide resections, and he died of systemic metastasis 22 months after the initial surgery. The second was a tumor, 2.2 x 2.0 x 1.5 cm in size, originating in the left seventh intercostal muscle of a woman 24 years of age. A metastatic tumor occurred in the same muscle, and wide resection including the seventh and eighth intercostal muscles was performed. There has been no recurrence for 1.5 years since surgery. Metastasis of chest wall epithelioid sarcoma in the muscle in which the primary lesion resides should be considered, even if the primary lesion is small.     

Malignant rhabdoid tumor of the vulva: is distinction from epithelioid sarcoma possible? A pathologic and immunohistochemical study

Epithelioid sarcoma (ES) and malignant rhabdoid tumor (MRT) have heretofore been regarded as two separate clinicopathologic entities. However, they have some histologic similarities, and both represent histogenetic and phenotypic enigmas. This study reports the pathologic and immunohistochemical findings of four vulvar neoplasms occurring in young women that represented diagnostic dilemmas because of their similarity to both ES and MRT. Only one case had the classic histologic features of ES, whereas, in our opinion, the other three cases fulfilled the histologic criteria of MRT, despite the fact that two of the three cases were reported earlier as examples of ES. Neither electron microscopy nor immunohistochemistry has been found to be helpful in separating ES from MRT, mainly because they share several ultrastructural and immunophenotypic features. The behavior of these vulvar tumors--ours and the few published examples of ES--is generally aggressive, more in keeping with MRT than classic ES. We believe that some, if not most, putative ES of the vulva are in fact MRT, a neoplasm with an unfavorable prognosis.     

Extremity epithelioid sarcoma. Amputation vs local resection.

Amputation has traditionally been advised for extremity epithelioid sarcoma because of its pattern of innocuous presentation and relentless soft-tissue and nodal metastasis. To assess the role of amputation in extremity epithelioid sarcoma, we reviewed our experience with 42 patients treated between 1961 and 1986. On presentation with localized primary tumor (n = 18), nine of 11 patients who underwent wide local excision and four of six patients who underwent excisional biopsy were free of disease, and one patient who underwent amputation died. After presentation with localized recurrence (n = 12), four of six patients who underwent wide local excision and two patients who underwent excisional biopsy were free of disease; three other patients who underwent wide local excision had margins that tested positive on pathologic examination, of whom one was free of disease; one patient who underwent amputation died of disease. On presentation with regional metastasis (n = 12), only one of five patients who underwent wide local excision and one of seven patients who underwent amputation were free of disease. Primary amputation offered no apparent overall survival benefit to patients presenting with regional metastasis. The favorable outcome after local resections for localized disease indicates that wide local excision with margins that test negative on pathologic examination is preferable to radical amputation in these patients.     

Undifferentiated carcinoma of the vulva mimicking epithelioid sarcoma.

We report an undifferentiated sweat gland carcinoma of the vulva in an 80-year-old woman. The tumor, which was located in the right labium majus, resembled an epithelioid sarcoma histologically; it had a granulomatous appearance with multiple tumor nodules containing epithelioid tumor cells. The tumor also contained rhabdoid cells; a large cluster of them showed histological features indistinguishable from those of a malignant rhabdoid tumor. Immunohistochemically, the tumor cells reacted not only for epithelial markers such as cytokeratins, EMA, and CEA, which are known to be expressed by epithelioid sarcoma, but also for CA125 and with monoclonal antibodies recognizing sweat gland structures--namely, EKH5 and EKH6. For comparison, two epithelioid sarcomas and two extrarenal malignant rhabdoid tumors were also studied. Of these tumors, only one extrarenal rhabdoid tumor reacted with EKH5, and none reacted for CA125. Electron-microscopic examination of the present tumor showed the presence of discontinuous basal laminae and tonofibril-like structures as well as primitive cell junctions and interdigitating filopodia. From these findings, we conclude that the tumor was an undifferentiated sweat gland carcinoma mimicking an epithelioid sarcoma. Findings in this case support the idea of the diverse histogenesis of extrarenal malignant rhabdoid tumors and indicate that electron microscopy is important for differentiating epithelioid sarcoma from skin adnexal carcinoma.     

Immunoproliferative small-intestinal disease. An immunohistochemical study

We performed a detailed histological and immunohistological study on both fresh-frozen and paraffin-embedded tissue from full-thickness jejunal biopsy specimens taken from three patients with immunoproliferative small-intestinal disease (IPSID). In all three patients, the mucosal infiltrate consisted of "centrocyte-like" (CCL) cells forming lymphoepithelial lesions and plasma cells. In one patient, the mucosal infiltrate was strikingly follicular. Immunohistochemistry showed alpha 1 heavy chain, but no light chain, in the perinuclear space and cytoplasm of the CCL cells and in the plasma cells. In two patients, the plasma cells (but not the CCL cells) also contained alpha 2 heavy chain. In the case showing a follicular pattern, the extrafollicular CCL cells and most of the cells within the mucosal follicles expressed alpha 1 heavy chain, but a minor and variable population of cells expressed polytypic IgM. The dendritic reticulum cells stained for alpha 1 (but not alpha 2) heavy chain, mu chain, and both light chains. In all cases, the CCL cells did not stain for common acute lymphoblastic leukaemia antigen (CALLA); in the follicles, CALLA negative cells displaced a residual CALLA-positive population to the periphery and merged with the CALLA negative cells outside the follicles. These findings confirm the homology between IPSID and low-grade B-cell "Western" lymphomas arising in mucosa-associated lymphoid tissue; they suggest that the follicular pattern sometimes seen in these lymphomas is caused by selective colonization of reactive follicles by CCL tumor cells.     

上皮样肉瘤的症状和治疗

目的报道5例手部上皮样肉瘤(epithelioid sarcoma)并结合国内外29篇文献介绍其临床表现，治疗方法及预后。方法5例肿瘤均在手掌部，皮肤无异常，伴不适感或轻度压痛。4例的受累指伸直受限，呈轻度屈曲状。肿瘤的长径为1．0—5．0cm，质地硬韧，边界不清，底部固定。均行肿瘤边缘切除术。结果病理诊断为上皮样肉瘤。2例在术后2个月内复发，1例作截肢术，1例拒绝手术。3例术后随访3-18个月，无复发。结论上皮样肉瘤好发于青少年及手部，初起时无明显症状，为手部常见软组织恶性肿瘤之一。无特异性症状及体征，国内用肿瘤边缘切除术和扩大切除术，国外用根治术。诊断依靠病理结果，术后复发率高，预后不佳。     

Proliferative myositis. An immunohistochemical and ultrastructural study

We studied four cases of proliferative myositis by the avidin-biotin-peroxidase complex technique, using a panel of 12 antibodies, and by electron microscopy. The aim was to clarify the nature of their constituent cells, specifically the giant ganglion-like cells and spindle cells, and to discuss the implications for histogenesis. In all cases, both cell types showed positive cytoplasmic staining with antibodies to vimentin, actin (C4), and alpha-smooth muscle actin-1, but in only one was there positive staining with desmin. No staining was obtained with factor XIIIa, muramidase, alpha-1-antitrypsin, myoglobin, S-100 protein, CAM 5.2, factor VIII-related antigen, or neuron-specific enolase. By electron microscopy, both types of cells were seen to contain numerous thin filaments, dense bodies, coated and pinocytotic vesicles, active and dilated rough endoplasmic reticulum, few microvilli, and incomplete desmosomal junctions. Our findings imply a myofibroblastic nature for the giant ganglion-like cells and spindle cells. Our observations also support the hypothesis that they are derived from a pericytic cell.     

Intra-articular epithelioid sarcoma showing mixed classic and proximal-type features: report of 2 cases, with immunohistochemical and molecular cytogenetic INI-1 study

Epithelioid sarcoma, a rare sarcoma with epithelial differentiation, most often occurs in the distal extremities; however, it may occur in essentially any location. With the recent recognition that the loss of expression of the tumor-suppressor gene INI-1 may be associated with epithelioid sarcoma, it has become clear that epithelioid sarcoma may occur in previously unsuspected locations such as bone. Only 2 cases of intra-articular epithelioid sarcoma have been previously reported. We retrieved 2 intra-articular cases coded as epithelioid sarcoma from our archives. Both expressed cytokeratins (AE1/AE3 and OSCAR), CD34, vimentin, and epithelial membrane antigen, and showed complete loss of expression of INI-1. Fluorescence in situ hybridization was performed on formalin-fixed, paraffin-embedded sections by using a laboratory-developed dual-color probe containing INI1 (CTD-2511E13 and CTD-2034E7) (22q11.2) (OR) and PANX2 (RPCI3-402G11) (22q13.33) (GR) probes as control. Both cases occurred in a clearly intra-articular location in the knee. Case 1 was that of a 19-year-old man with a long-standing history of pain and limited joint function. This patient was disease free after amputation. Case 2 was that of a 60-year-old woman. Follow-up information available for this patient showed bilateral subpleural metastases. Morphologically, case 1 showed features of proximal-type epithelioid sarcoma, whereas case 2 showed mixed features of classic and proximal-type epithelioid sarcoma. Immunohistochemistry showed complete loss of INI-1 protein in both cases; fluorescence in situ hybridization analyses were negative for INI-1 gene deletion. Herein, we have reported 2 cases of intra-articular epithelioid sarcoma, showing morphologic and immunohistochemical features identical to those of epithelioid sarcoma in conventional locations, including loss of INI-1 expression. Intra-articular epithelioid sarcoma should be distinguished from malignant pigmented villonodular synovitis and from carcinoma metastatic to the synovium. Improved recognition of this rare clinical presentation should allow for better understanding of its unique features.     

Cystic pulmonary metastases from epithelioid cell sarcoma

A patient with cystic pulmonary metastases, from epithelioid sarcoma of the thigh, resulting in bilateral pneumothoraces is presented. The pulmonary metastases superficially resembled pneumomatoceles and were not visible on radiography. Computed tomography examinations over a 10-month period showed no change in the size or number of the cystic metastases, though air fluid levels and pericystic nodular consolidation (probably due to pulmonary hemorrhage) did occur and regress. This case report serves to emphasize that bullous type lesions on thoracic computed tomography in patients with a known sarcoma should be interpreted with particular caution.     

Filiform large-cell lymphomas. An ultrastructural and immunohistochemical study

Ten cases of "undifferentiated" large-cell tumors were ultrastructurally characterized by cells with abundant filiform cytoplasmic projections without intercellular junctions. These cases were studied by means of the avidin-biotin-peroxidase complex (ABC) technique applied to formalin-fixed, paraffin-embedded sections using antibodies against high- and low-molecular weight keratins (Ker), vimentin (Vi), epithelial membrane antigen (EMA), S-100 protein, leucocyte common antigen (LCA), kappa (K) and lambda (L) light chains, Leu-M1, lysozyme (Ly), alpha-1 antitrypsin (A1AT) and alpha-1 antichymotrypsin (A1ACT). All 10 cases were negative for Ker and EMA but positive for Vi. S-100 was present only in scattered dendritic cells. LCA was identified in seven cases. In the three LCA-negative cases, two stained for Leu-M1, and one of these also showed intracytoplasmic L; one was negative for all markers but Vi. None of the tumors showed any significant staining for Ly, A1AT, or A1ACT. Our findings indicate that these tumors are nonepithelial and nonneuroectodermal, and that they are best classified as non-Hodgkin's lymphomas. The possibility that some of the filiform large-cell lymphomas may be derived from dendritic reticular cells cannot be excluded.