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PubMed1977

Ultrastructure of a cardiac rhabdomyoma.

Electron microscopic study of a cardiac rhabdomyoma removed at open heart operation revealed large rounded or polygonal cells that contained large amounts of monoparticulate glycogen. Myofibrils in these cells were few and located either subjacent to the plasma membranes or radiating from central areas. Leptofibrils and masses of anomalous Z band material were abundant. Shallow tubular sarcolemmal invaginations and elements of free and junctional sarcoplasmic reticulum were associated with the myofibrils. Desmosomes and nexuses were evident in intercellular junctions, which were extensive and randomly distributed throughout the cell surfaces. It is concluded that cardiac rhabdomyomas probably are hamartomas.

Cell Membrane↗
PubMed1986

Production of basement membrane laminin and type IV collagen by tumors of striated muscle: an immunohistochemical study of rhabdomyosarcomas of different histologic types and a benign vaginal rhabdomyoma.

Immunohistochemical methods were used to demonstrate the distribution of basement membrane laminin and type IV collagen in eight tumors derived from striated muscle (three botryoid, two alveolar, and two adult-type rhabdomyosarcomas; one benign vaginal rhabdomyoma). All of the tumors produced significant amounts of both basement membrane components. Stainings clearly revealed the alveolar nature of the rhabdomyosarcomas, with the alveolar spaces surrounded by distinct basement membranes. Different stages of cellular development were identified in the botryoid sarcomas, with the most immature cells of the cambium layer devoid of external basement membrane around the tumor cells, although the stroma contained finely dispersed basement membrane material and some cells contained intracytoplasmic laminin or type IV collagen, indicative of the synthesis of these proteins. The more mature cells, which had abundant granular cytoplasm, were enveloped by distinct basement membranes and seemed to have coalesced, forming structures resembling myotubes. The adult-type rhabdomyosarcomas were composed of large pleomorphic cells that were surrounded by basement membranes, either individually or in small groups. Some giant cells contained intracytoplasmic laminin. The vaginal rhabdomyoma was composed of round rhabdoblastic cells or elongated strap cells with cross-striations. Cells of both of these types were surrounded by thin but distinct basement membranes. The results suggest that demonstration of basement membranes would be helpful in the diagnosis of tumors derived from striated muscle. The findings concerning different stages of maturation of tumor cells are in accordance with previous in vitro observations of myoblastic cells.

Adult↗
PubMed2000

Spermatic cord rhabdomyoma.

A 67-year-old man presented with a scrotal swelling. Intraoperatively, this swelling was found to arise from the spermatic cord structures and was completely excised. Histopathology revealed a rhabdomyoma arising from the cord. The patient has had no evidence of recurrence in the past 24 months. We present the various types of rhabdomyomas and discuss the differential diagnosis for a spermatic cord swelling.

Aged↗
PubMed1999

Rhabdomyoma of the true vocal fold.

Rhabdomyomas of the larynx are exceedingly rare. The incidence, sites of occurrence in the larynx, and treatment of these benign striated muscle tumors are reviewed. A case of rhabdomyoma involving the vocalis muscle is presented with magnetic resonance imaging and video-stroboscopic documentation.

Aged↗
PubMed1991

Sublingual adult rhabdomyoma. Report of a case.

A case of intraoral adult extracardiac rhabdomyoma is reported. There has been no recurrence in 4 years follow-up. We estimate this to be the 111th reported case of extracardiac rhabdomyoma, the 29th to present in the tongue and sublingual regions.

Cell Nucleus↗
PubMed1975

Rhabdomyoma purum of the larynx.

A case of pure well-differentiated rhabdomyoma of the larynx occurring in a 50-year-old male is reported. The lesion was localized to the interarytenoid region. From an accurate revision of the world literature on the subject it appears that the cases so far described number only seven, including the one in the present report. Furthermore, the differential histological and histochemical criteria for distinguishing rhabdomyoma from granular cell Schwannoma are discussed and tabulated, as the two neoplasms may be easily confused. In our case, there has been no evidence of recurrence of the tumour for more than 1 year, which is in accordance with the previous cases reported in the literature.

Adult↗
PubMed1976

Rhabdomyoma of the hypopharynx and larynx. Report of two cases and a review of the literature.

Two cases of extracardiac rhabdomyoma are reported in a 54-year-old man with unexplained difficulty in breathing and in a 39-year-old man with a prolonged history of hoarseness. Attention is drawn to the necessity of doing hypopharyngo-and laryngoscopy in such cases. One tumour was found in the left vallecula in the hypopharynx and the other on the left vocal cord. The literature and the histological findings in 53 cases are reviewed. It appears that this rare tumour is most frequently found in muscles derived from the pharyngeal arches. A total of 40 cases have been diagnosed during the last decade, and this increase is likely to continue in the future. It may turn out that extra-cardiac rhabdomyoma is not so rare as was first presumed.

Adult↗
PubMed1986

Rhabdomyoma of the larynx.

Since 1897 a total of 37 cases of extracardiac rhabdomyomas of the fetal type have been reported in the literature. Only two of these were localized in the larynx. We report an additional case of extracardiac rhabdomyoma localized in the larynx, and the pertinent literature is discussed. Although rare, its existence should be kept in mind in the differential diagnosis of laryngeal tumours.

Aged↗
PubMed1994

Rhabdomyoma of the larynx: a rare cause of stridor.

Rhabdomyomas of the larynx are rare tumours. In the literature to date 18 cases have been reported. They usually present with slowly progressive symptoms typical of their benign nature. We present a case that has many atypical features for an 'adult' type rhabdomyoma and discuss the relevant associated literature.

Female↗
PubMed1999

Multiple rhabdomyomas in monozygotic twins.

Rhabdomyoma is the most frequent primary cardiac tumor seen during infancy. We report multiple rhabdomyomas diagnosed in twins in the neo natal period. To the best of our knowledge, this is the first instance of this happening.

Echocardiography, Doppler↗
PubMed2000

Pre-excitation syndrome secondary to cardiac rhabdomyomas in tuberous sclerosis.

Rhabdomyomas are not uncommon in infants with tuberous sclerosis. We describe a neonate who presented with hydrops fetalis arising from a tachyarrhythmia during fetal life related to rhabdomyomas. After reversion of the arrhythmia, pre-excitation was noted on an interval electrocardiogram. Following regression of the tumours, the delta wave disappeared with no further arrhythmias noted.

Cesarean Section↗
PubMed1992

[Differential diagnosis of cervical space occupying lesion: the parapharyngeal rhabdomyoma].

A case of a parapharyngeal rhabdomyoma is presented. Only about 30 cases of this benign tumor of striated muscles are reported in the literature. Most tumors were located in the lateral cervical region. Histologically a rhabdomyoma must be distinguished from other mesenchymal tumors, especially from granular cell myoblastoma, hibernoma and reticulohistiocytoma. The value of magnetic resonance imaging in the radiological evaluation of parapharyngeal masses is emphasized. Operative resection is generally regarded as the therapy of choice. Local recurrences have been reported in some cases; malignant degeneration is not known.

Contrast Media↗
PubMed1993

Crystal-storing histiocytosis associated with lymphoplasmacytic neoplasms. Report of three cases mimicking adult rhabdomyoma.

Massive crystal deposition is rare in lymphoplasmacytic (LPc) or plasma cell neoplasms. We report three cases in which the accumulation of crystals in histiocytes closely reproduced the histologic features of adult rhabdomyoma. The patients, all female, aged 18, 77, and 78 years, presented with tumor of cervical lymph nodes (two cases) or the otolaryngic mucosa (two cases). In addition, two patients had monoclonal serum or urine immunoglobulin (IgM-kappa-1, unknown-1), and one had renal and bone marrow involvement on biopsy. This last patient died of acute renal failure at 5 months, another was alive without disease at 8 years, and the remaining one was lost to follow-up. Lymph nodes, mucosae, and kidney showed a neoplastic LPc infiltrate masked by sheets of large benign histiocytes containing sheaves of crystals. Paraffin-section immunohistochemistry demonstrated monoclonal staining of the LPc cells in all cases (IgM-kappa-2, IgA-kappa-1) and of the crystals (IgM-kappa) in one case. In all patients, the crystal-containing cells were positive for KP-1 (CD68), but not for desmin, muscle-specific actin, or myoglobin. These findings suggest that, in any case of adult rhabdomyoma in which the histologic findings are not typical, a crystal-storing histiocytosis should be ruled out: recognition of the atypical LPc component and the histiocytic immunophenotype of the crystal-storing cells will help prevent a serious misdiagnosis.

Adolescent↗
PubMed1978

Extracardiac rhabdomyoma: a rare entity.

Extracardiac rhabdomyomas are rare benign lesions of striated muscle origin. A review of the world literature revealed about 27 acceptable cases of benign, adult-type rhabdomyoma with a distinct male predominance. In most of the reported cases, the tumor was located in the head and neck region, usually submucosal or deeper. The case reported here is unusual because the tumor was subcutaneously located in the neck. The tumor was fairly well circumscribed, multilobulated, and easily shelled out. Light microscopy showed large round to elongated cells with granular, highly eosinophilic cytoplasm, often with peripheral vacuolation. Cross striations were infrequent. The nuclei were often peripheral and had prominent nucleoli. Electron microscopic studies confirmed the tumor's myogenic origin. Myofibrils with Z band material, abundant mitochondria, and glycogen particles were observed.

Diagnosis, Differential↗
PubMed1996

Neural variant of fetal rhabdomyoma and naevoid basal cell carcinoma syndrome.

A 15-year-old boy with the characteristic features of the naevoid basal cell carcinoma syndrome presented with a retroperitoneal mass. The tumour showed morphological features of a still ill-defined variant of fetal rhabdomyoma, characterized by well-differentiated nerve fibres admixed with immature striated muscle cells, similar to neuromuscular choristoma. Four cases of fetal rhabdomyoma and naevoid basal cell carcinoma syndrome have been previously reported. The behaviour of this tumour has been benign, although a complete excision was impossible due to its close relation with abdominal vascular trunks.

Adolescent↗
PubMed2006

Cardiac rhabdomyoma in a neonate: application of serial echocardiography.

Primary cardiac neoplasms are rare. Rhabdomyoma is the most common benign congenital tumor found in infancy and has a tendency for spontaneous regression. We report a case of a cardiac rhabdomyoma in a symptomatic neonate in whom serial echocardiography was used for diagnosis and documentation of initial regression as early as 1 month.

Diagnosis, Differential↗
PubMed1982

A case of adult rhabdomyoma in the tonsillar region. A light and electron microscope study.

A case of rhabdomyoma in a 71-year-old woman is reported. The lobulated, soft tumour was situated in the left tonsillar region. It was encapsulated, easily removable and of a uniformly greyish-red colour on the cut surface. Both light and electron microscopy showed the classical features of a benign rhabdomyoma of adult type, with large "spider" cells and typical myofilaments, as well as Z-band fragments. To achieve the correct diagnosis in such cases the most important thing is probably to bear the possibility of this rare, but benign tumour in mind.

Aged↗