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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of an intrathoracic, extrapleural, infantile rhabdomyofibrosarcoma in a 4-year-old boy. Histologically, the primary lesion showed extensive hyalinization and stroma sclerosis and was composed of relatively uniform spindle-shaped, at least focally rather polygonal tumour cells with scattered intracytoplasmatic globoid inclusions. Although chemo- and radiotherapy was given postoperatively, local recurrences and metastases in the lung and thymus have developed; the patient died of tumour disease 3 years later. Recurrences and metastases showed features of tumour progression with higher cellularity and increased mitotic activity. Immunohistochemically, the tumour cells stained strongly positive for vimentin, desmin, and muscle-specific actin, and at least focally for MyoD1; the tumour did not stain for alpha-smooth muscle actin, neural and epithelial markers, or CD34 and CD31. The differential diagnosis of these aggressive tumours in the spectrum of spindle-cell lesions in early childhood is discussed.
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PMID:[Infantile rhabdomyofibrosarcoma. An aggressive tumor in the spectrum of spindle cell tumors in childhood]. 892 96

We report 19 unusual cases of mixed tumors and myoepitheliomas arising in soft tissues. The neoplasms occurred in 12 males and seven females. The age at diagnosis ranged from 2 to 83 years (mean 35, median 30). Eight tumors arose in the upper limb, six in the lower limb, three in the trunk, and two in the head and neck region. Three cases involved both dermis and subcutis; the remainder arose in subcutaneous (13 cases) or deep subfascial soft tissue (three cases). The most common presenting complaint was a painless swelling, with duration ranging from 2 weeks to 1 year (median 2.5 months). Microscopically, the tumors were predominantly well circumscribed and lobulated. Six cases showed a focally infiltrative margin. Cardinal morphologic features included nests, cords, and ductules of epithelioid cells and/or nests of spindled cells within a hyalinized to chondromyxoid stroma. One tumor was predominantly composed of myoepithelial cells and devoid of epithelial differentiation (i.e., ductules). Cytoplasmic hyaline inclusions were noted in two cases; squamous differentiation was seen in one case. Osteoid production and/or metaplastic bone was observed in three tumors. Chondroid differentiation (usually mature) was seen in four cases. Adipocytic differentiation was seen in two tumors. Mitotic activity was variable but generally scant; atypical mitotic figures were not identified. By immunohistochemistry, 16 of 16 cases expressed pan-keratin; 16 of 17 S-100 protein; six of 14 alpha smooth muscle actin (IA4); two of 10 muscle specific actin (HHF-35); two of 10 desmin; three of 11 glial fibrillary acidic protein; and three of 16 epithelial membrane antigen. Clinical follow-up was available in 10 patients and ranged from 6 months to 20 years (mean 4.25 years, median 2 years). Two patients developed local recurrence; metastasis to lung and lymph nodes were observed in two additional patients. Both of the latter patients died. We believe that these findings expand the concept of cutaneous mixed tumors to include neoplasms composed predominantly of myoepithelial cells and to include tumors arising in deeper subcutaneous and/or subfascial tissues. The clinical behavior of such neoplasms, when arising in soft tissues, may be difficult to predict but is most often benign; however, a minority of lesions metastasize. Until larger studies with longer follow-up are available, treatment and prognostication are probably best based on criteria used in comparable salivary gland tumors.
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PMID:Mixed tumors and myoepitheliomas of soft tissue: a clinicopathologic study of 19 cases with a unifying concept. 963 Jan 86

Sex cord-stromal tumors of the pediatric testis present diagnostic and therapeutic challenges. This study examines the clinicopathologic features of 16 testicular sex cord-stromal tumors from children less than 18 years of age. Four juvenile granulosa cell tumors and five tumors of Sertoli or incomplete differentiation in this study had high mitotic rates and/or sarcomatoid areas that suggested malignancy, but none of these children developed recurrence or metastases. Some of these tumors had been initially misdiagnosed as yolk sac tumors or rhabdomyosarcomas because of the presence of areas superficially resembling these neoplasms. These morphologic pitfalls have received little attention in the literature. Even incompletely differentiated sex cord-stromal tumors have at least focal areas characteristic of juvenile granulosa or Sertoli cell differentiation. In addition, immunohistochemical negativity for alpha-fetoprotein, muscle specific actin, and desmin are useful for ruling out yolk sac tumor and rhabdomyosarcoma. Four patients had Leydig cell tumors and three had large cell calcifying Sertoli cell tumors. Children with Leydig cell tumors are not at risk for metastasis, but children with large cell calcifying Sertoli cell tumors are at risk for endocrine syndromes as illustrated by one of our cases. The differential diagnosis of these tumors is also discussed.
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PMID:Testicular sex cord-stromal tumors in children: clinicopathologic study of sixteen children with review of the literature. 902 45

Desmoplastic small round cell tumor (DSRCT) typically occurs in the abdomen but may also present at other sites. We report six cases of paratesticular DSRCT. The patients, who ranged in age from 17 to 37 (mean, 28) years, presented with a scrotal mass (five cases) or testicular pain (one case). Grossly, the tumors were white to tan and firm. Typically, they involved the paratesticular soft tissue, serosal surfaces and the epididymis near the junction with the rete testis. Microscopically, the tumors consisted of nests of mitotically active "small blue cells" with scant cytoplasm embedded in a densely fibrotic stroma. Two tumors showed focal tubule formation; one of these also formed rosettes. The tumors exhibited the typical immunophenotype of DSRCT (positivity for keratin, vimentin, desmin, and neuron-specific enolase but nonreactivity with HBA-71 and anti-S-100). Four tumors metastasized to lymph nodes (retroperitoneal, cervical, and two unspecified); pulmonary metastases occurred in one of these cases and in one patient without lymph node metastases. One of the above patients treated with chemotherapy, died of disease at 16 months. The patients with pulmonary metastases (one of whom also had lymph node metastases) were treated with aggressive chemotherapy and are alive and apparently disease-free at 2.5 and 3 years, respectively. Three of the six patients, two of whom had known metastases, were lost to follow-up. The DSRCT of the paratestis has histologic and immunohistochemical features identical to its abdominal counterpart and must be differentiated from other "small blue cell" tumors of the paratesticular region.
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PMID:Desmoplastic small round cell tumors of the paratesticular region. A report of six cases. 904 90

Primitive neuroectodermal tumors (PNET) of the female genital tract are rare and more common in the ovary, but uncommon in the cervix uteri. A 26-year-old woman presented with suspect cervical smears. The conization specimen showed a small cell non-keratinised squamous cell carcinoma with involved margins. The patient underwent radical abdominal hysterectomy and pelvic lymphonodectomy. The microscopic examination showed a densely cellular tumor of small neuroendocrine cells with scanty cytoplasm and rosettes. Immunohistochemically, the cells were slightly positive for NSE and negative for S 100, GFAP, neurofilaments, squamous cell cytokeratin 1, vimentin, desmin and leukocyte common antigen. The diagnosis of PNET, stage pT1b1,N0, M0 was made. The patient underwent adjuvant pelvic radiation. Three years later, pulmonary metastases occured. Radiation therapy of the thorax and six courses of combination chemotherapy (5-FU and cis-platinium) could not prevent tumor progression. The patient died 4.2 years after diagnosis. The autopsy showed widespread lymphatic metastases and hepatic, pulmonal and skeletal metastases and a peritoneal carcinosis. The tumors are resistent to radio- and chemotherapy, and the prognosis is generally poor. Up to 15% foci of squamous or glandular differentiation occur in or adjacent to these tumors. So the authors favor the histogenesis from a pluripotent endocervical stem cell. The neuroendocrine component of mixed tumors improve the prognosis. Therefore, it is necessary to recognize this component.
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PMID:Primitive neuroectodermal tumor of the cervix uteri. A case report. 906 88

We studied 18 patients with primary malignant smooth muscle tumors of the lung (7 women and 11 men, 5-76 yr old, with a mean age of 50 yr). Lesions varied from 1.7 to 10 cm in greatest diameter. The tumors were classified as low (4 cases), intermediate (2), or high grade (12). Low-grade lesions were characterized by an orderly proliferation of fascicles of spindle cells that intersected at right angles and showed oval-to-spindle cells with cigar-shaped nuclei, minimal pleomorphism, and low mitotic activity, without hemorrhage or necrosis. Intermediate-grade lesions retained the fascicular configuration but showed increased cellularity with atypia and dense chromatin pattern, occasional pleomorphism, and mild increase in mitotic activity. High-grade lesions showed high cellularity, marked pleomorphism and atypia, frequent areas of hemorrhage and necrosis, and high mitotic activity. Immunohistochemical studies in 16 cases showed positive staining of tumor cells with smooth muscle actin in 12, desmin in 5, and coexpression of actin and/or desmin and keratin in 3. Six patients with low- and intermediate-grade lesions were alive and well from 2 to 12 years after diagnosis (mean follow-up time, 6 yr); 8 with high-grade lesions died of their tumors with widespread metastases from 1 to 24 months after diagnosis (median survival time, 5 mo). One patient whose tumor showed features of high-grade leiomyosarcoma was alive and well 12 years after surgery. Three patients with high-grade tumors were lost to follow-up. Our findings suggest that histologic grade may be the most reliable prognostic parameter for predicting clinical behavior in primary leiomyosarcoma of the lung and that smooth muscle actin is the most sensitive immunohistochemical marker for establishing the diagnosis in these tumors. Primary leiomyosarcoma should be considered in the differential diagnosis of pulmonary spindle cell neoplasms; histologic grading may be of value in the planning of therapy and assessment of prognosis for these lesions.
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PMID:Primary leiomyosarcomas of the lung: a clinicopathologic and immunohistochemical study of 18 cases. 912 17

A case of myofibrosarcoma of the breast is described. A 55-year-old woman presented with a small mammary nodule. A large recurrent lesions appeared a month later, and the patient died 11 months following initial presentation from diffuse pleuropulmonary metastases. Histologically, the primary tumor consisted mainly of spindled cells, arranged in fascicles and surrounded by varying quantities of dense hyaline collagen. The recurrent lesion had a more pleomorphic organization. In both lesions, there was positive immunostaining for vimentin, smooth-muscle actin, and fibronectin, and negative results for desmin, laminin, and type IV collagen. Electron microscopy revealed abundant rough endoplasmic reticulum, myofilaments with focal densities, and the fibronexus junctions and fibronectin fibrils characteristic of myofibroblasts. Given these cellular features and behavior, the tumor was interpreted as a malignant neoplasm showing myofibroblastic differentiation, i.e., a myofibrosarcoma. This case enlarges the group of myofibrosarcoma of breast, also with the demonstration of fibronexus and fibronectin fibrils. The paper emphasizes the criteria required for myofibroblastic differentiation and reviews lesions of the breast reported in the literature as myofibroblastic.
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PMID:Myofibrosarcoma of the breast: review of the literature on myofibroblastic tumors and criteria for defining myofibroblastic differentiation. 913 Sep 98

Cutaneous malignant melanomas in cats, both melanotic and amelanotic, were diagnosed in 57 of 1.530 skin tumors during the period 1991-1995. All melanomas occurred in domestic shorthaircats of ages 3-19 years (mean = 11.5 years). Postmortem examination was performed on 16 cats. All had metastases in the regional lymph node and several organ systems. The average time of survival after surgical removal of the tumor was 4.5 months. Histologically, five types of melanomas could be distinguished: epithelioid, spindle, mixed, signet-ring, and balloon cell. Whereas all epithelioid, spindle, and mixed epithelioid/spindle cell types showed pigmentation, signet-ring and balloon cell types were often amelanotic. Immunohistochemical examination of the melanomas revealed a positive staining for S-100, vimentin, and neuron-specific enolase. The melanomas were negative for muscle cell markers, except in some of the signet-ring cell melanomas; 13 of 21 tumors showed a weak positive staining for polyclonal desmin. Electron microscopic examination of signet-ring cell melanomas revealed an abundance of intermediate filaments, whereas in some of these tumors a few cells with melanosomes were found. Nonisotopic in situ hybridization for mRNA encoding for tyrosinase verified the melanocytic origin of the amelanotic signet-ring and balloon cell melanomas.
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PMID:Cutaneous malignant melanomas in 57 cats: identification of (amelanotic) signet-ring and balloon cell types and verification of their origin by immunohistochemistry, electron microscopy, and in situ hybridization. 915 May 43

We present the clinical, light microscopic, and immunohistochemical features of two new cases of leiomyosarcoma of the female breast. Both the patients were old (83 and 86 years) and were referred with a history of a long-standing breast lump. The results of the physical examination and the preoperative radiologic investigations suggested a phyllodes tumor. The patients were treated with mastectomy. The tumors measured 6 and 6.5 cm in the largest dimension, respectively, and were composed of fascicles of atypical, actively proliferating spindle cells, often intersecting at right angles. The axillary lymph nodes were free of tumor. Immunohistochemically, the tumor cells were positive for desmin, muscle-specific actin, and vimentin and negative for other markers, including keratins and hormone receptors. Focal rhabdomyoblastic differentiation was noted in one case. Follow up at 1 year is negative for metastases or local recurrences. Our study confirms that leiomyosarcoma of the breast is a locally invasive neoplasm and that it could represent a peculiar anatomic entity among mesenchymal tumors of the breast.
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PMID:Leiomyosarcoma of the female breast: report of two new cases and a review of the literature. 957 87

We report the clinical, histopathologic, immunohistologic, and prognostic findings in 19 patients with cutaneous leiomyosarcoma, eight males and 11 females (mean age, 66 years; age range, 41-93 years). The tumors presented mainly as solitary lesions and were located on the head and neck (eight lesions), trunk (four lesions), upper extremities (three lesions), and lower extremities (four lesions). Histopathologically, two predominant growth patterns were observed: nodular (12 cases) and diffuse (seven cases). Neoplasms with a nodular growth pattern were characterized by high cellularity and prominent nuclear atypia, and they showed conspicuous mitoses, several necrotic cells, and sometimes extensive necrotic areas. By contrast, most cutaneous leiomyosarcomas with a diffuse growth pattern revealed low cellularity, well-differentiated smooth muscle cells, inconspicuous mitotic figures, and few or no necrotic cells. Immunohistologic investigations revealed all cutaneous leiomyosarcomas to express vimentin and smooth muscle actin. Pan-muscle actin (HHF-35) was also expressed in most cases (15 lesions). However, only 12 lesions showed positive staining for desmin. Remarkable was the expression of cytokeratins in five lesions. Clinical follow-up revealed local recurrences in five patients (three cases with nodular pattern and two lesions with a diffuse pattern) after a period ranging from 8 months to 3 years after surgical excision. No distant metastases have been observed in our series. We conclude that cutaneous leiomyosarcoma with a diffuse growth pattern may constitute a pitfall in histopathologic diagnosis because of the presence of only subtle criteria for malignancy. Cutaneous leiomyosarcoma may show different immunophenotypes, thus emphasizing the importance of using a large panel of antibodies (smooth muscle actin, HHF-35, desmin, vimentin, cytokeratins, and S-100 protein) in immunohistologic diagnosis. Cutaneous leiomyosarcoma sometimes reveals local recurrences, but it has negligible potential for distant metastases.
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PMID:Cutaneous leiomyosarcoma. 929 73


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