Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients with rhabdomyosarcoma presented clinically with dermal nodules on the face. The first patient was a 12-month-old girl with a 2 X 3-cm-diameter red nodule on her left cheek that had begun as a small red papule when she was 3 months old and had progressively increased in size. The second patient was a 19-year-old girl who presented with an enlarging mass on her right cheek that had begun as a pea-sized nodule three months previously. In both cases, biopsies revealed a rhabdomyosarcoma in the underlying soft tissue with extension into the overlying dermis. Rhabdomyosarcoma presenting as a dermal nodule is rare. It usually presents as an asymptomatic papule without distinctive clinical features and therefore may result in delayed diagnosis unless a biopsy is performed.
Arch Dermatol 1988 Nov
PMID:Rhabdomyosarcoma presenting as a cutaneous nodule. 246 31

Congenital alveolar rhabdomyosarcoma in a newborn was reported. She had multiple subcutaneous tumors and looked like a blueberry muffin baby. From the clinical signs and the histological pictures, a diagnosis of neuroblastoma was considered. Laboratory data showed no abnormalities. Electron microscopic examination revealed myofilament and incipient stria formation. On the basis of these data, a final diagnosis of congenital alveolar rhabdomyosarcoma associated with multiple skin metastases was made.
J Dermatol 1989 Oct
PMID:Congenital alveolar rhabdomyosarcoma presenting as a blueberry muffin baby. 260 Feb 81

A generalized ichthyosiform eruption developed in a 13-year-old boy three weeks before presentation with a paravertebral rhabdomyosarcoma. His skin returned to normal shortly after the onset of chemotherapy and remission of the tumor. This case is unusual because the ichthyosis developed acutely, following a generalized urticarial eruption, and also involved the palms and soles.
Pediatr Dermatol 1988 Aug
PMID:Acquired ichthyosis in a child with rhabdomyosarcoma. 320 56

Embryonal rhabdomyosarcoma is the most frequent of tissue sarcomas in children. Its location in the subcutaneous tissue makes it a dermatological diagnosis. In childhood, the exact histological type of the tumour, sometimes difficult to determine, is absolutely necessary since prognosis and treatment differ according to the histogenetic form. Rhabdomyosarcoma is rare in adults. One must rule out malignant pleomorphic histiocytoma which has a more favourable prognosis. Among the antisera recently made available, those directed against desmin, foetal skeletal myosin and/or specific skeletal muscle myofilament seem to be most useful when associated with the anti-myoglobin antibody.
Ann Dermatol Venereol 1988
PMID:[Subcutaneous rhabdomyosarcoma in children. Clinical, immunologic and ultrastructural aspects]. 322 87

A 3-year-old girl had a large exophytic mass protruding from the nares, of seven months' duration. Routine microscopic studies demonstrated a tumor composed of solid sheets of small cells, with scattered foci lining alveola-like spaces. Immunohistochemical studies demonstrated the presence of desmin and myoglobin, and the absence of prekeratin, neuron-specific enolase, and leukocyte common antigen. These observations are consistent with the diagnosis of alveolar rhabdomyosarcoma.
Pediatr Dermatol 1988 Nov
PMID:Alveolar rhabdomyosarcoma arising in the nasal cavity of a 3-year-old child. 323 83

Beckwith-Wiedemann syndrome, familial atypical multiple mole melanoma syndrome, and hereditary tylosis are bona fide genodermatoses with malignant potential. Each of these conditions is associated with an increased incidence of certain tumors: Wilms' tumor, adrenocortical carcinomas, pancreatoblastomas, and hepatoblastomas in Beckwith-Wiedemann syndrome; intraocular malignant melanoma, pancreatic carcinoma, and noncolorectal gastrointestinal cancers in familial atypical multiple mole melanoma syndrome; and squamous cell carcinoma of the esophagus in hereditary tylosis. Other cancer-related genodermatoses are Birt-Hogg-Dube syndrome (associated with medullary carcinoma of the thyroid and renal cell carcinoma) and its variant, Hornstein-Knickenberg syndrome (associated with colon carcinoma). Kidney tumors (Wilms' tumor and malignant rhabdoid tumor), leukemias (acute myelogenous and acute myelomonocytic), retinoblastoma, and paratesticular rhabdomyosarcoma have been reported recently in children with another genodermatosis-incontinentia pigmenti. Supernumerary nipples (polythelia) may be sporadic or familial in occurrence; their presence has been associated with an increased incidence of renal adenocarcinoma, testicular cancer, prostate cancer, and urinary bladder carcinoma. The general characteristics, mucosal and skin manifestations, and noncutaneous features of all these conditions are reviewed. Also, the associated malignancies of these genodermatoses and other conditions that are characterized by dermatologic manifestations and may be either familial or secondary to an inherited gene defect are summarized.
Dermatol Clin 1995 Jan
PMID:Miscellaneous genodermatoses: Beckwith-Wiedemann syndrome, Birt-Hogg-Dube syndrome, familial atypical multiple mole melanoma syndrome, hereditary tylosis, incontinentia pigmenti, and supernumerary nipples. 771 45

We describe a patient with rhabdomyosarcoma of the posterior cervical region and clear cell sarcoma on the occipital scalp. These two tumors later metastasized to distant skin. We differentiated these tumors by histopathologic, histochemical, immunohistochemical, and ultrastructural findings. Cells of the posterior cervical tumor showed differentiation toward striated muscle, whereas those of the occipital tumor showed findings compatible with melanocytic differentiation.
J Am Acad Dermatol 1994 Nov
PMID:A patient with rhabdomyosarcoma and clear cell sarcoma of the skin. 796 39

The frequency of different malignant cutaneous tumors (MCTs), primary and metastatic, in children is not known. We reviewed all MCTs, primary and metastatic, seen during a 20-year period in a large general pediatric hospital. Fifty-three MCTs, 36 primary and 17 metastatic, were diagnosed in 36,207 pediatric dermatology patients. The incidence was 1.4 per 1000 patients. The relative frequency of occurrence of the different tumors was as follows: rhabdomyosarcoma, 25%; lymphomas, 19%; basal cell carcinoma, 13%; leukemia, 13%; neuroblastoma, 10%; malignant melanoma, 6%; squamous cell carcinoma, 6%; unclassified sarcomas, 4%; epithelioid schwannoma, 2%; ependymoma, 2%. The mean follow-up was 3 years; 48% died, 27% were lost to follow-up, and 25% are under control. We conclude that primary and metastatic MCTs in children are rare. Their types differ from MCTs in an older age population. MCTs in children are associated with a high mortality rate, often related to late recognition.
J Am Acad Dermatol 1994 Feb
PMID:Malignant cutaneous tumors in children. Twenty years of experience at a large pediatric hospital. 828 84

Patients with vitiligo have been found to have circulating antibodies to pigment cells. To evaluate the functional activity of these antibodies, a highly sensitive europium release assay was used to compare complement-mediated cytolysis of human melanocytes by sera of 56 patients with vitiligo (20 with active disease, 25 with inactive disease, 11 with unidentified disease activity) and 47 control individuals. Significant melanocyte lysis was mediated by 32 (57%) of the patients with vitiligo but by only three (6%) of the control sera (p < 0.001), and by 17 (85%) of 20 patients with active vitiligo versus 11 (44%) of 25 patients with inactive disease (p < 0.025). Mean melanocyte lysis by vitiligo sera was 24% versus 6% by control sera (p < 0.0001). A subset of 12 vitiligo sera with high titers of cytolytic antibodies to melanocytes (34% mean cytolysis) reacted minimally (< 2% mean cytolysis) to a panel of control cells that included human and murine melanomas, human fibroblasts, lung carcinoma, and rhabdomyosarcoma. These findings indicate that antibodies present in patients with vitiligo have the functional ability to selectively kill melanocytes and are more common in active disease. These observations support, but do not prove, the hypothesis that vitiligo is an autoimmune disease and that anti-pigment cell antibodies have a role in inducing the disease.
J Invest Dermatol 1993 Jun
PMID:Cytolytic antibodies to melanocytes in vitiligo. 849 21

Rhabdomyosarcoma (RMS), a high-grade, malignant, skeletal muscle tumor, represents approximately 5% of neoplasms in children. The poorly differentiated forms of RMS are often not easily diagnosed and classified. Among the four histologic variants, alveolar RMS is the least frequently reported subtype. A poorly differentiated solid variant of alveolar RMS occurred on the right hand of a 16-year-old girl. Because of the tumor size, local invasiveness, and occurrence of cutaneous and breast metastases at presentation, the clinical staging was group IV (T2/NO/M1). Surgical excisions of the primary and metastatic locations were performed and chemotherapy with vincristine, dactinomycin, cyclophosphamide, and doxorubicin was administered. Light and electron microscopy studies revealed a solid proliferation with a focal alveolar pattern of monomorphous, small, round neoplastic cells without easily detectable muscular morphologic features. The skeletal muscle origin was revealed by the positive immunostaining for desmin, alpha-sarcomeric actin, muscle-specific actins, and enolase, and confirmed by immunoblotting for desmin. Despite the age of our patient, which is considered by some authors an independent predictor of outcome, all prognostic variables were unfavorable. However, a disease-free interval during three years of follow-up underlines the importance of multidisciplinary regimens for the treatment of this rare solid tumor of childhood and adolescence.
Pediatr Dermatol 1995 Dec
PMID:Solid alveolar rhabdomyosarcoma of the hand in adolescence: a clinical, histologic, immunologic, and ultrastructural study. 874 83


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