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At least 973 records · Page 54Linked to original sources
PubMed2001

Serum creatine kinase levels parallel the clinical course for rhabdomyomatous Wilms tumor.

A right-sided renal mass in an 11-month-old girl was diagnosed by percutaneous needle biopsy as Wilms tumor, which on histologic examination was found to be predominantly rhabdomyomatous. As part of the examination, serum creatine kinase (CK) and CK-MB levels were measured and were significantly elevated at 994 U/L (reference range, 42-180 U/L) and 40 U/L (reference range, 0-3 U/L), respectively. Subsequently, an 8-month-old girl was admitted to the hospital with septicemia and was found to have an abdominal mass. A diagnosis of bilateral Wilms tumor was made following percutaneous biopsy of both kidneys; histologic examination confirmed that the tumor was predominantly rhabdomyomatous. Serum CK and CK-MB levels also were measured and were significantly elevated at 685 U/L and 84.4 U/L, respectively. In both cases, the serum CK and CK-MB levels reflected the clinical course; elevation in serum levels was associated with tumor recurrence, infarction, or chemotherapy-related necrosis. We conclude that these enzymes have clinical usefulness as markers for Wilms tumor showing rhabdomyomatous morphologic features.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2003

Rhabdomyomatous multilocular thymic cyst.

The thymus is the seat of a diverse array of pathologic conditions given its embryologic roots. Multilocular thymic cysts, although well described, are uncommon, and one associated with rhabdomyomatous elements has not been described previously. A 15-year-old boy complained of sudden-onset chest pain of a month's duration, but was otherwise well. Chest radiographs localized the mass to the anterior mediastinum, arising from the thymus. A computed tomography scan demonstrated the lesion to be a multilocular fluid-containing cyst. A large, 15-cm cyst contiguous with the thymus was removed. Histologic evaluation confirmed a multilocular cyst lined mainly by mucinous epithelium with focal areas of ciliated and squamous lining. A prominent finding was skeletal muscle elements in the form of spider cells, strap-like cells, and foci reminiscent of fetal-type muscle with cross-striations. At the periphery of the cyst, thymic tissue with branching ducts and Hassall corpuscles were noted. No evidence of skin and/or its appendage structures, cartilage, or other differentiated tissue was seen despite generous sampling of the specimen. The muscle elements, most likely, were derived from thymic myoid cells, while the multilocular cyst arose from remnants of the thymomedullary system.

Adolescent↗
PubMed1985

[Pathological findings of cardiac rhabdomyomatosis in the guinea pig].

Cardiac rhabdomyomatosis of Hartley guinea pigs was examined by light and transmission electron microscopy, and its incidence and distribution were also described. Cardiac rhabdomyomatosis was found in 58 cases out of 345 animals, aged from 1.5 to 17 weeks. A few small lesions were noted in many cases, but large lesions were rare. Most lesions noted in the ventricle were found to measure from 1 mm2 to 5 mm2 in area. The larger lesions were seen near the cardiac apex. In terms of distribution of the lesions, they were observed most frequently in the left ventricular free wall. They were also distributed less frequently in the ventricular septum and in the right ventricular free wall. In bilateral free walls and the ventricular septum, this lesion was frequently noted on the endocardial side and in the tunica muscularis, respectively. Light microscopically, this lesion had a characteristic nodular-reticular structure with large vacuolated cells. Electron microscopy of cardiac rhabdomyomatosis revealed two cell types, A and B. Type A cells were characterized by large aggregates of glycogen particles distributed loosely in the cytoplasm and by myofibrils and mitochondria located at the periphery of the cytoplasm. Type B cells were characterized by large aggregates of mitochondria near the nucleus and at the periphery of the cytoplasm and by large aggregates of glycogen particles with a similar distribution to those in type in type A cells. Myocardial cells in the marginal regions of rhabdomyomatosis lesions showed mitochondrial swelling and rupture of myofibrils.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMedDate not supplied

Rhabdomyomatosis of newborn lung: a case report with immunohistochemical and electronmicroscopic characterization of striated muscle cells in the lung.

Interstitial proliferation of striated muscle cells in the lung is rare. A few cases in the literature only document infants with associated lung and other major organ anomalies incompatible with long-term survival. In this report we document a case of diffuse patchy interstitial proliferation of striated muscle cells in the left lung and confirm their nature by immunohistochemistry and electron microscopy. In particular, we show that these skeletal muscle cells express alpha-sarcomeric actin, but not alpha-smooth muscle actin, indicating an antigenetically well-developed striated muscle phenotype despite morphologically embryonal features.

Female↗
PubMedDate not supplied

Rhabdomyomatous mesenchymal hamartoma of skin.

A distinctive dermal and subcutaneous hamartoma composed primarily of disorganized skeletal muscle and mature adipose tissue, designated rhabdomyomatous mesenchymal hamartoma of skin, was recently reported. We have observed two histologically similar though less polypoid lesions in boys aged 4 years. Both were also on the chin and present since birth, suggesting that site and age, as well as histology, may be characteristic. The location on the chin in three of the four neonates may reflect an etiology of aberrant embryologic development of the platysma muscle as it inserts superficially into the dermis over the mandible.

Child, Preschool↗
PubMed1975

Tuberose sclerosis with malignant astrocytoma.

A case is described of a recurrent malignant gemistocytic astrocytoma in which a diagnosis of tuberose sclerosis was made at post-mortem examination, when "rhabdomyomata" were found in the cardiac muscle and tubers in the cerebral hemispheres. Malignant degeneration of a cerebral lesion has not been described in association with tuberose sclerosis.

Astrocytoma↗
PubMed1982

Cardiac tumour. An echocardiographic diagnosis.

The clinical and echocardiographic features in nine patients with cardiac tumours are reviewed. In two patients, tumours were diagnosed by means of M-mode echocardiography only and in seven by means of both two-dimensional and M-mode echocardiography. The diagnosis was not made clinically in any of the nine patients. Cardiac catheterisation performed in four patients resulted in a diagnosis of mitral valve disease in two and a correct diagnosis in the other two. A definite diagnosis of cardiac tumor was not possible with M-mode echocardiography alone in three patients. A correct diagnosis was made in all seven patients in whom M-mode and two-dimensional echocardiography were performed.

Adolescent↗