[Rhabdomyomatous mesenchymal hamartoma].

Grupo de Investigación en Patología Estructural, Funcional y Clínica, Universidad Industrial de Santander, Bucaramanga, Colombia.
Actas Dermo-Sifiliográficas 08/2008; 99(6):474-6. DOI: 10.1016/S1578-2190(08)70292-0
Source: PubMed

ABSTRACT Rhabdomyomatous mesenchymal hamartoma is an extremely rare congenital lesion, and very few cases have been reported even though its macroscopic and microscopic features make diagnosis easy. An 18-year-old woman consulted with a pedunculated mass in the medial region of her neck. The mass was surgically removed, and rhabdomyomatous mesenchymal hamartoma was diagnosed. The clinical, macroscopic, histologic, and immunochemical characteristics that allow diagnosis of this entity are discussed. Although association with congenital abnormalities is uncommon, this possibility should be assessed by the clinician.

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    ABSTRACT: We describe the location, size, histopathologic aspect and immunohistochemical expression of a rhabdomyomatous mesenchymal hamartoma, with the aim of providing useful information for its correct diagnosis. A 31-year-old Chinese man first presented 2 years previously with a solitary subcutaneous mass on the left side of his neck and under his mastoid process; the mass's size was 2x2cm. The mass increased in the size in the past 2 years. Magnetic resonance imaging revealed a dumbbell shaped and well-outlined highly reflective mass, with its upperpart infiltrating the interspace of the atlanto-occipital joint. The mass was surgically removed. On macroscopic examination, the mass was oblong and partly encapsulated, the size of the mass was 4.9x3.5x3cm, and its cut side was grey. On histologic examination, it showed a disordered collection of bundles of mature striated muscle fibres arranged in a haphazard manner and interspersed with adipose tissue, fibrocytes or mesenchymocytes and collagen, and had a myxoid matrix. On immunochemical examination, mature striated muscle was positive for desmin and myoglobin, adipose tissue and nerves were positive for S-100 protein, and fibrocytes or mesenchymocytes and collagen were positive for vimentin and cluster of differentiation 34. A diagnosis of rhabdomyomatous mesenchymal hamartoma was established. Rhabdomyomatous mesenchymal hamartoma is a rare dermal or subcutaneous lesion, and we describe its immunohistochemical expression for the first time. This case report provides more information on the microscopic appearance and immunohistochemical expression.
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