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Multiple Glomangiomyoma Versus Glomangioma

2000; Lippincott Williams & Wilkins; Volume: 22; Issue: 4 Linguagem: Inglês

10.1097/00000372-200008000-00015

1533-0311

Carlos Monteagudo, Carmen Cardá, Antonio Llombart‐Bosch, Luís Calduch, Esperanza Jordá,

Soft tissue tumor case studies

To the Editor: Regarding the interesting paper from Yang et al. (1) on congenital multiple plaque-like glomangiomyoma, we would like to make some conceptual comments and discuss our own observations on the light and electron microscopic features of a case of multiple disseminated glomangiomas/glomangiomyomas. In 1924, Pierre Masson (2,3) described the neuromyoarterial glomus, which he later renamed the neurovascular glomus, and its tumors. The term glomangioma was coined by Bailey (4) in 1935 and is currently applied to those cases having wide vascular lumina, which are most commonly found in patients with multiple tumors. The term glomangiomyoma was introduced for those cases in which conventional spindled smooth muscle cells are seen in gradual transition with typical glomus cells (5). This subtype represents about 10% of all glomus tumors but nearly 20% of the lesions in cases of multiple tumors (5,6). In this regard, however, we have to note that Masson originally distinguished two regions within glomus tumors: type 1 and type 2 vessels. The former represent a short transition between the afferent artery and type 2 vessels, and they are characterized by several layers of usual smooth muscle cells surrounded by polygonal glomus cells. In contrast, no internal conventional smooth muscle component is found in type 2 vessels, where glomus cells are progressively reduced in number as they approach efferent venules (3). Hence, in Masson's view, conventional smooth muscle cells may be considered a constant, although usually minor, component of glomus tumors. In addition, ultrastructural studies of solitary and multiple lesions have shown that glomus cells have smooth muscle features such as cytoplasmic myofilaments, dense bodies, subplasmalemmal plaques, basal lamina, and prominent pinocytosis (7–11). For this reason, although it was initially believed to be a pericyte, most authors currently consider the glomus cell to be a smooth muscle cell variant. Interestingly, a cutaneous epithelioid angioleiomyoma has been reported recently, in which a differential diagnosis with a glomus tumor proved extremely difficult (12). We have studied several lesions from a case of familial congenital disseminated multiple glomangiomas by light and electron microscopy. Thirty-five lesions were located in the extremities, trunk, face, and neck of a 33-year-old woman. Histologically, areas of conventional glomangioma, with a few layers of epithelioid glomus cells surrounding cavernous vascular spaces, were found to contain a variable number of spindle cells (Fig. 1). Two of the lesions were ultrastructurally analyzed, showing polygonal glomus cells with abundant cytoplasmic myofilaments, dense bodies, and pinocytotic vesicles (Fig. 2a and b). These cells acquired an elongated shape and the myofilaments were more organized and parallel to the long cellular axis (see Fig. 2c). As a proof of their contractile ability, wavy cellular and nuclear contours were seen (see Fig. 2c). These findings are in accordance with previous ultrastructural studies reporting the presence of myoid features in glomus cells (7–11) and the detection of spindled smooth muscle cells within these tumors (8,10).FIG. 1.: (A) Low-power view of one of the lesions exhibiting wide vascular lumina. (B) Higher magnification showing glomus cells and a few spindle cells.Figure 1: ContinuedFIG. 2.: Ultrastructural findings. (A) Several layers of polygonal glomus cells. (B) Higher magnification of glomus cells showing myofilaments, dense plaques, and basement membrane. (C) Progressive transition from glomus cells to conventional spindled smooth muscle cells (original magnification ×2,500 [a]; ×6,000 [b]; ×10,000 [c]).Figure 2: ContinuedFigure 2: ContinuedIn summary, according to Masson's original descriptions, subsequent ultrastructural work, and our own findings, we believe that both terms, glomangioma and glomangiomyoma, actually designate the same lesion, with only subtle quantitative differences. Moreover, if extensive sampling is done, transitional areas from typical glomus cells to well-defined conventional smooth muscle cells can be found in multiple lesions, in our experience, even in the absence of florid histologic features of glomangiomyoma. Carlos Monteagudo M.D. Carmen Carda M.D. Antonio Llombart-Bosch M.D. Luis Calduch M.D. Esperanza Jordá M.D.