Granular cell myoblastoma of the tongue.
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Cogenital smooth muscle hamartoma is a congenital skin lesion characterized by proliferation of bundles of smooth muscle within the reticular dermis. We report on a group of 15 children with this lesion, confirmed by skin biopsy specimen, its prevalence, the occurrence of associated anomalies, and long- term follow-up. To our knowledge, this is the largest group of patients with this lesion described in the literature. The estimated prevalence is about 1:2600 live births with slight male predominance. The lesion is most frequent in the lumbosacral area (67% [10/15]) and a positive pseudo-Darier's sign is present in 80% (12/15) of patients. During the follow-up period of up to 7 years, the lesions enlarged slightly but became less prominent. No malignant transformation was observed.
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We present five cases with gastro (four cases) and intestinal (one case) myogenic tumors with a marked extraluminal growth. In all cases, incidental discovery of an asymptomatic mass prompted further examination. One of three cases with a pedunculated growth mimicked a gallbladder cancer. The mass of a jejunal leiomyoma case changed markedly in location under probe compression. Color Doppler sonography confirmed not only the hypervascular nature of the mass but also feeding and draining vessels.
Inflammatory myofibroblastic tumors, also called inflammatory pseudotumors, are rare pseudosarcomatous proliferations. Their behavior is generally not aggressive, but in most instances they can simulate malignant neoplasms, from which they are impossible to distinguish before excision. One case of myofibroblastic tumor of the spleen in a 5-year-old boy is described: the patient was treated with a partial splenectomy, which was found to be the best approach for diagnosis and treatment. The purpose of this report is to call attention to this entity with peculiar histologic and immunohistochemical characteristics, which has to be included in the differential diagnosis of splenic lesions and may be excised by a nonaggressive procedure.
Malignant fibrous histiocytoma (MFH) and inflammatory myofibroblastic tumor (IMT) are uncommon primary non-epithelial cell tumors of the pancreas. In addition, there are inflammatory pseudotumors (IPT) that may arise in the course of autoimmune pancreatitis (AIP). In the English language literature, only 24 cases of IMT and nine cases of MFH in the pancreas have been reported to date. We investigated three individual spindle cell tumors of the pancreas that were identified as MFH, IMT, and IPT, respectively, using immunohistochemical and molecular analysis. Both the MFH and the IMT, but not the IPT, showed nuclear p53 expression and mutations of the p53 gene. The MFH and the IMT also had higher mitotic and Ki-67 (MIB-1) indexes than the IPT. The IPT was found to be a tumor-like case of AIP. Many IgG4-positive plasma cells, which are considered to be a feature of AIP, were found in all three tumors. It is concluded that in this series of spindle cell tumors of the pancreas, apart from immunohistochemical features, the demonstration of p53 mutations may be helpful in distinguishing true neoplastic tumors from pseudotumors such as IPTs arising in the context of AIP.
The clinical and pathologic features of congenital granular-cell myoblastoma in five infant girls are reported. One lesion, treated expectantly, progressively decreased in size and after 3 yr and 9 mo could not be detected, while two lesions which were imcompletely excised did not recur. It is suggested that congenital granular-cell myoblastoma is caused by an intrauterine stimulus, and that this stimulus may possible be production of estrogen by the fetus. Congential granular-cell myoblastoma should be treated expectantly or by limited excision, and has an excellent prognosis.
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A seventy-year-old man with a painless, perineal neoplasm was found to have a granular cell myoblastoma densely adherent to the bulbar urethra. This case represents the fourth known perineal neoplasm of neural origin.
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The clinical and radiological features and endoscopic findings in 13 patients with smooth muscle tumours of the upper gastrointestinal tract are reviewed. The study emphasises the non-specific nature of the symptomatology and exemplifies a number of aspects of the radiological picture of these disorders. It also illustrates some of the problems that can arise in their investigation, by radiological and endoscopic methods, and gives some indication of the favourable prognosis that may obtain when complete surgical removal of the tumour is possible.