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Fine needle aspiration in myxoid tumors of the soft tissues.

Myxoid tumors of soft tissues constitute a histogenetically heterogeneous group of lesions, both benign and malignant, that show an overproduction of mucopolysaccharide substances. A correlative fine needle aspiration (FNA) cytologic and histologic study was performed on 16 such cases (3 intramuscular myxomas, 2 lipoblastomas, 6 myxoid liposarcomas, 4 myxoid malignant fibrous histocytomas [MFHs] and 1 extraskeletal myxoid chondrosarcoma), with electron microscopic examination of the aspirate in 4 cases. FNA of all tumors produced a gelatinous material. Smears from all but one of the tumors had a granular myxoid background substance that stained blue to blue-red with the Diff-Quik stain; the exception was the extraskeletal myxoid chondrosarcoma, whose smears stained intensely blue-red to red. On light microscopy, only lipomatous tumors had distinctive cells with diagnostic value: the lipoblasts. Although the arrangement of lipoblasts in lipoblastomas differed somewhat from that found in myxoid liposarcomas, clinical data were important for making a definitive diagnosis. Intramuscular myxomas generally showed a low cellularity composed of fibroblastlike cells with no cytologic atypia. Myxoid MFHs were composed of fibroblastlike cells and macrophagelike cells; the elongated cells with slight-to-moderate nuclear atypia predominated in tumors of low-grade malignancy, while polygonal or round cells with one or more nuclei and marked nuclear atypia predominated in high-grade tumors. Although extraskeletal myxoid chondrosarcoma was mainly differentiated from low-grade myxoid MFH by its metachromatic matrix, ultrastructural studies showed the distinctive distended cisternae with microtubular aggregates in the extraskeletal myxoid chondrosarcoma, thus contributing to the definitive diagnosis.

Adult↗

Sclerosing stromal tumor of the ovary in a premenarchal female.

Sclerosing stromal tumor (SST) is a rare benign ovarian neoplasm of stromal origin with less than 100 cases reported in the literature. Unlike the other stromal tumors, thecomas and fibromas, which tend to occur in the fifth and sixth decades, sclerosing stromal tumors predominantly affect females in the second and third decades. Computed tomography (CT), magnetic resonance imaging (MRI), and ultrasound findings have been described, but have not been reported previously in the pediatric literature. We present a case of SST of the ovary in a 10-year-old premenarchal female, the youngest patient to our knowledge reported in the literature, and describe the ultrasound and CT findings with pathologic correlation.

Child↗

Ectomesenchymal chondromyxoid tumour of the tongue: Unlikely to originate from myoepithelial cells.

Ectomesenchymal chondromyxoid tumour is a recently described rare entity found almost exclusively in the anterior dorsum of the tongue. We report an additional case, review the pertinent literature and explore the histogenesis of the lesion. The lesion has no discriminating clinical features and diagnosis is only established following histopathological examination including immunohistochemical assessment. The histogenesis of the lesion remains speculative and our study seems to discount myoepithelial cells as a potential source.

Biomarkers, Tumor↗

[Pathology of primary tumors and pseudotumors of the pleura].

Primary tumors are relatively rare in the pleura. Histological types include mesothelioma, epitheliod, biphasic or sarcomatoid tumors as well as primary lymphoma and mesenchymatous tumors which include solitary fibrous tumor, epithelioid hemangioendothelioma and angiosarcoma and synovialosarcoma. We detail here the new WHO classification 2004 explaining the different entities, excluding metastatic tumors which are the most frequent tumors of the pleura.

Hemangioendothelioma, Epithelioid↗

Desmoplastic small round cell tumor of the pleura.

Three cases of desmoplastic small round cell tumor (DSRCT) with multiphenotypic differentiation, primary in the pleura, are presented. This is a previously unrecognized site for this tumor type. Two patients were male and one female aged 29, 24, and 17 years. All presented with chest pain and were found to have pleural-based tumors associated with pleural effusion. Abdominal involvement was not present in any of the cases. Histologically, the tumor showed the characteristic features of intra-abdominal DSRCT, including angulated nests of small cells embedded in a vascular fibroblastic stroma, focal rhabdoid phenotype, and areas of central necrosis. The neoplastic cells showed evidence of epithelial, mesenchymal, and neural differentiation with characteristic dot-like positivity for vimentin and desmin topographically corresponding to perinuclear aggregates of intermediate filaments identified on electron microscopy in one case. Two patients died of disease 2 years and 15 months after presentation, respectively, and one patient is alive with disease 18 months after presentation. The histogenesis of DSRCT is unknown. Most previously reported cases involved the peritoneum or tunica vaginalis, suggesting a histogenetic relationship to the mesothelium. The occurrence of these tumors in the pleura lends further support to this theory.

Adolescent↗

Collision tumor of the stomach: a case report of mixed gastrointestinal stromal tumor and adenocarcinoma.

Collision tumors of the stomach are uncommon. To the best of our knowledge, this is the first case report of gastric collision tumor composed of gastrointestinal stromal tumor (GIST) intermixed with primary adenocarcinoma in the English literature. The adenocarcinoma was determined to be the primary tumor based on histologic features. The tumor cells of the GIST were diffusely and strongly positive for CD34 and CD117, weakly positive for smooth muscle actin (5% of cells), and negative for desmin, S-100 protein, synaptophysin, and cytokeratin. There was no transition between the different components. We hypothesized that the stomach was influenced by the same unknown carcinogen, resulting in a simultaneous proliferation of different cell lines (epithelial and stromal cell). This case represents an example of two independent tumors in a unique one-on-another pattern, namely growth of adenocarcinoma on GIST.

Adenocarcinoma↗

Absence of Epstein-Barr virus (EBV) in a gastrointestinal stromal cell tumour (GIST) in an adult human immunodeficiency virus-seropositive patient with past EBV infection.

Gastrointestinal stromal cell tumours (GIST) of the small intestine are rare malignancies. Recently, an association of Epstein-Barr virus (EBV) with malignant stromal cell tumour in young people with AIDS and past EBV infection has been described. We describe a 33-year-old heterosexual male with asymptomatic human immunodeficiency virus (HIV) infection who had had an EBV infection in the past and who presented with an EBV-negative GIST. The association between EBV and malignant stromal cell tumours in young people with AIDS could not be reconfirmed in our adult patient. The relationship between EBV and malignant stromal cell tumours in AIDS patients and the possible pathogenetic role of EBV remains to be established, at least in adults.

Adult↗

Malignant gastric stromal tumor: unusual metastatic patterns.

We present the case of a woman with metastatic malignant gastric stromal tumor occurring four years following a partial gastrectomy and distal esophagectomy, which presented as atypical breast mass with synchronous occult gastrointestinal bleeding. A discussion of metastatic pattern is presented, with emphasis placed on the need for continued surveillance after resectional surgery for gastrointestinal stromal tumor (GIST).

Breast Neoplasms↗

Cellular digital fibromas: distinctive CD34-positive lesions that may mimic dermatofibrosarcoma protuberans.

BACKGROUND: Digital fibromas are common benign acral tumors typically reported as angiofibromas (AFs) or acquired digital fibrokeratomas (ADFs). Cellular variants are not well recognized. METHODS: We collected 14 acral fibrocytic lesions showing a spindle cell morphology from our files, and evaluated CD34, Factor XIIIa, epithelial membrane antigen (EMA), and S100 protein staining of these lesions. We compared the histologic and immunohistochemical features of these cellular fibromas with five digital AFs, five ADFs, and five digital dermatofibromas. RESULTS: The 14 cellular digital fibromas showed intersecting fascicles of thin delicate bland spindle cells in the superficial reticular dermis with a fibrotic-to-slight myxoid stroma. The spindle cells in all cases stained strongly for CD34, and only scattered stromal cells stained for Factor XIIIa. Five tested cases were negative for EMA and S100 protein. The digital AFs, fibrokeratomas, and dermatofibromas stained predominately for Factor XIIIa, with no or minimal staining for CD34. CONCLUSIONS: These findings suggest that a subset of digital fibromas is characterized by a dense cellular proliferation of CD34-positive spindle cells. Awareness of this variant of digital fibroma and its staining pattern is critical in preventing misdiagnosis as dermatofibrosarcoma protuberans, particularly in superficial biopsies.

Adult↗

Papular elastorrhexis in childhood improved by intralesional injections of triamcinolone.

Papular elastorrhexis is a rare disease developing asymptomatic skin-colored small papules in adolescence with histopathological loss of elastic fibers. There has been no established treatment for this disease. A 4-year-old Korean boy had multiple, hard, whitish papules on his chest and back for one year. Histopathologic examination revealed focal loss of elastic fibers in the dermis, and X-ray examination showed no bony abnormalities. His skin lesions were improved by intralesional injections of triamcinolone but recurred after four months.

Back↗

Sclerosing stromal tumours of the ovary.

Sclerosing stromal tumours of the ovary have recently been described as a histologically and clinically distinct subgroup within the thecoma-fibroma spectrum of benign ovarian sex cord stromal tumours. Reported cases occurred predominantly in young women and only occasional tumours showed evidence of hormonal activity. The present series of five cases expands the spectra of both histological patterns and clinical presentations and suggests that the entity of sclerosing stromal tumours may not be as clearly circumscribed as has been previously reported.

Adolescent↗