Alveolar soft-part sarcoma. A study of 13 typical examples and one with a histologically atypical component

Thirteen cases of typical alveolar soft-part sarcoma and one in which the tumor had a histologically atypical component are presented. The atypical zone in the latter case was characterized by increased nuclear hyperchromatism and pleomorphism, increased nuclear-cytoplasmic ratio, decreased cytoplasmic eosinophilia and granularity, a less distinct "nesting" pattern, and more numerous mitotic figures. The tumors occurred almost entirely in the younger decades and involved a variety of soft tissue sites. Sex distribution was 1:1, and there was a slight left-sided predominance in tumor location. Median survival of the patients with typical alveolar soft-part sarcoma was 79 months (the patient with the atypical neoplasm died at 36 months). The major factor related to both survival and the likelihood of metastasis was tumor size.     

Alveolar soft-part sarcoma responding to interferon alpha-2b

A 23-year-old woman with an alveolar soft-part sarcoma of her calf with pulmonary metastases unresponsive to chemotherapy is described. Interferon (IFN) alpha-2b induced an impressive tumour response still ongoing after IFN treatment had to be stopped because of a psychosis. An explanation of this effect is still speculative.     

Kaposi's sarcoma: a clinico-pathologic overview.

A careful overview of the classical appearances of Kaposi's sarcoma (KS) as well as of its variants were reviewed from the clinical and pathological point of view. The growth phases (stages) and the cellular patterns were histopathologically compared with emphasis on the developmental progression of disease as well as mitotic activity. Other morphological aspects were also assessed such as the features of the early phases and the incidence of hyaline bodies. One hundred and forty-three lesions from 96 patients mostly of the Italian sporadic type were investigated. A complete list of those entities which should be considered in differential diagnosis is shown and the dilemma of whether KS is a neoplasia or a hyperplasia is discussed.     

Alveolar soft part sarcoma. A cytologic and immunohistochemical study

Alveolar soft part sarcoma is an uncommon soft tissue tumor that has seldom been studied by cytologic methods. The cytomorphologic features of two cases of this sarcoma are described. To enhance diagnostic accuracy in a suspected alveolar soft part sarcoma, the authors present the differential diagnosis and the application of immunocytochemical procedures to cytologic specimens.     

Alveolar soft part sarcoma. a report of 15 cases

The aim of this study was to evaluate the presentation, course and treatment outcome of 15 patients with this rare type of sarcoma. The files of the patients were retrospectively analysed. Overall survival was calculated according to the Kaplan-Meier method. There were 15 patients, 8 male and 7 female. The mean age at diagnosis was 29 years for men and 24 years for women. The median survival was 48 months with an overall 5-year survival of 38%. 5 patients had haematogenic metastases at the time of diagnosis. For the remaining 10 patients with localised disease, the median survival was 48 months and the 5-year survival 48%. The median disease-free survival for these patients was 12 months with a 5-year disease free survival of 40%. After the occurrence of haematogenic metastases, patients survived a median period of 8 months (range 0-45 months). 5 patients are still free of disease after a median period of 234 months (12-295 months). Alveolar soft part sarcoma is found especially in young adults. When diagnosed, it is often metastasised with a poor prognosis. However, when radically resected, long-term survival is possible.     

Central neurocytoma. A clinico-pathologic study of five cases.

Five cases of central neurocytomas are described. The tumors occurred in relatively young patients (range 14 to 43 years; mean age, 27) with no predilection for sex. All the lesions were located in the anterior portion of the lateral ventricles or in the third ventricle, involving the septum pellicidum or the fornix. Histologically, they were composed of uniform cells with round nuclei and clear cytoplasm resembling oligodendrogliomas or, to a lesser extent, ependymomas. In 4 tumors, protein cell nuclear antigen immunostaining showed a low cell proliferation rate. All cases were positive for neuron-specific enolase. Four of the 5 cases were strongly immunoreactive for synaptophysin. The immunohistochemical data were consistent with neuronal differentiation. Resection was subtotal in 4 cases and total in one. Postoperative radiotherapy was given in only one case. The follow-up revealed a good prognosis: 4 patients were alive and had a long survival (from 2 to 8 years). Only one patient died after 14 months for causes unrelated to the neoplasm. The authors emphasize the importance of immunohistochemistry to recognize this benign intraventricular tumor.     

A clinico-pathologic study of clear-cell hepatocellular carcinoma

We reviewed the pathologic and clinical features of 7 cases of clear-cell hepatocellular carcinomas. Tumor cells had a typical clear, empty cytoplasm due to prominent accumulation of glycogen and lipid droplets, as demonstrated by ultrastructural study in one of our cases. Follow-up of the 7 patients did not confirm the better prognosis of this tumor reported by other investigators. The clinical course of our cases was more unfavorable than that of the other 130 cases of usual hepatocellular carcinomas treated at this Institute.     

Gene expression profiling of alveolar soft-part sarcoma (ASPS)

Background  

Alveolar soft-part sarcoma (ASPS) is an extremely rare, highly vascular soft tissue sarcoma affecting predominantly adolescents and young adults. In an attempt to gain insight into the pathobiology of this enigmatic tumor, we performed the first genome-wide gene expression profiling study.     

Genital human papilloma virus infection in males. A clinico-pathologic study.

From March 1987 to December 1988, 402 male sexual partners of 317 women with human papilloma virus (HPV) infection of the lower genital tract and 85 with HPV-associated cervical and/or vulvar intraepithelial neoplasia (CIN and/or VIN) were submitted to clinical examination and peniscopy. The latter was performed at a 6-15 X magnification after a 3 min exposure to 5% acetic acid solution. Visible lesions were biopsied. Thirty-one patients had clinical evidence of HPV infection in the glans, penile shaft or urethra, and 222 had peniscopic evidence of subclinical aceto-white lesions. Of 31 patients with clinical lesions, 11 showed also aceto-white subclinical lesions. Of 253 peniscopically positive males, 237 were biopsied and 191 of these were histologically ascertained. Three patients had penile intraepithelial neoplasia, one with clinical appearance of a Buschke-L?wenstein tumor. The incidence of HPV infection in male sexual partners of women affected by HPV infection of the lower genital tract associated or not with intraepithelial neoplasia is lower than expected. However, clinically negative males should not be considered disease free; in fact, 12 patients, negative at the first examination, showed histological evidence of HPV infection at subsequent controls. Therefore, follow-up of at least 6 months should be allowed to identify HPV bearing males. The reported low frequency of HPV infection may be due to the fact that the males may harbour the virus in the urethra, prostate or seminal vesicles or penis without any clinical evidence of disease. Although research for HPV-DNA in intraurethral and penile scraping material might be useful for diagnosis, peniscopy with a 5% acetic acid application remains the clinical test for evaluating HPV infection in males. The importance of peniscopy should be viewed with respect to the prevention of infection or reinfection of the female sexual partners, in addition to the specific diagnostic purpose in male patients.     

Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients.

Myeloid sarcoma (MS) is a rare neoplasm whose knowledge is largely based on case reports and/or technically dated contributions. Ninety-two MSs in adulthood with clinical data available were evaluated both morphologically and immunohistochemically. Seventy-four cases were also studied by fluorescent in situ hybridization on tissue sections and/or conventional karyotyping on bone marrow or peripheral blood. Histologically, 50% of the tumors were of the blastic type, 43.5% either monoblastic or myelomonocytic and 6.5% corresponded to different histotypes. CD68/KP1 was the most commonly expressed marker (100%), followed by myeloperoxidase (83.6%), CD117 (80.4%), CD99 (54.3%), CD68/PG-M1 (51%), CD34 (43.4%), terminal-deoxy-nucleotidyl-transferase (31.5%), CD56 (13%), CD61/linker for activation of T cells (2.2%), CD30 (2.2%) and CD4 (1.1%). Foci of plasmacytoid monocyte differentiation were observed in intestinal cases carrying inv16. Chromosomal aberrations were detected in about 54% of cases: monosomy 7(10.8%), trisomy 8(10.4%) and mixed lineage leukemia-splitting (8.5%) were the commonest abnormalities, whereas t(8;21) was rare (2.2%). The behavior was dramatic irrespective of presentation, age, sex, phenotype and cytogenetics. Most if not all, long survivors received bone-marrow transplantation. The present report expands the spectrum of our knowledge showing that MS has frequent monoblastic/myelomonocytic differentiation, displays distinctive phenotypic profile, carries chromosomal aberrations other than t(8;21), and requires supra-maximal therapy.     

Pancreatic metastasis of alveolar soft-part sarcoma: A case report and review?of the literature

Alveolar soft-part sarcoma is a rare tumour. Patients commonlypresent with distant metastases both at the time of diagnosis and latein the course of disease. We present a case of pancreatic metastasis,occurring more than six years after diagnosis. Treatment consistedin subtotal pancreatoduodenectomy with pylorus resection. Both specificpatterns of relapse and treatment opportunities of this uncommon featureare discussed.     

Alveolar soft part sarcoma of the vagina

We describe a case of primary alveolar soft part sarcoma presenting as a vaginal mass in a 27 year old woman and also review the literature on this type of tumor arising in the lower female genital tract. Histologically, most of the tumor exhibited poorly developed alveolar architecture, but anaplasia was absent. Although the mass was well circumscribed, a few tumor cells were seen focally within the peritumoral tissue. Treatment consisted of wide local excision followed by external radiation therapy. The patient is alive without evidence of disease 17 years after operation.     

Efficacy of trabectedin in patients with advanced or metastatic alveolar soft-part sarcoma

Background: Alveolar soft-part sarcoma (ASPS) is a rare sarcoma often occurring in young patients that is characterized by the unbalanced translocation der(17)t(X;17) (p11;q25). Although itusuallyshowsan indolent clinical course, the prognosis is usually poor in advanced disease. Since standard chemotherapy regimens used in soft-tissue sarcomas lack efficacy in ASPS, new therapeutic options are needed. We investigated the efficacy of trabectedin, which has demonstrated activity in a variety of cancer types including some of the most prevalent translocation-related sarcomas. Patients and Methods: 7 patients with metastatic or advanced ASPS treated with trabectedin in the Sarcoma Center Berlin-Brandenburg and the University Hospital of Greifswald were analyzed for median progression-free survival (mPFS), overall survival (OS), and therapy-related toxicity. Results: In 6 patients with documented disease progression, disease stabilization was reached with trabectedin; only 1 patient experienced progressive disease. The mPFS and OS were 7 months and 21 months, respectively, since the start of trabectedin treatment. Overall, no severe Common Toxicity Criteria (CTC) grade 3 or 4 toxicity was observed. Conclusions: The poor prognosis of patients with ASPS has so far been due to the unavailability of effective systemic treatments. Trabectedin can be considered the only currently registered drug with clinical activity in this disease.     

Alveolar soft part sarcoma of tongue base — A rare presentation of a rare tumor

Alveolar soft part sarcoma is a rare, aggressive malignancy of uncertain histologic origin and enigmatic clinical behaviour. It has a characteristic histopathological picture, with a propensity for vascular invasion and distant metastasis. We report a case of alveolar soft part sarcoma involving the tongue base in an adolescent female. She underwent laser assisted excision of the tongue base tumour followed by post-operative radiotherapy. The clinical presentation, histopathological picture, immunohistochemical & cytogenetic studies, radio-imaging, management protocols and prognosis of this tumor have been discussed.