Multifocal Glomangiomyomas in the Chest Wall of a Young Man

To the Editor.—Multiple glomangiomyomas are rare, and to my knowledge have not been previously reported in the intercostal spaces. A case of multiple glomangiomyomas was encountered in the chest wall of a 30-year-old man who presented with a 10-year history of pleuritic chest pain. His computed axial tomographic scan showed a large left-sided mass along the posterior wall of the upper chest. Two other smaller, pleural-based masses were noted in the left hemithorax, 1 posteriorly and 1 laterally. At surgery, the chest cavity was entered at the left fourth intercostal space, and a large posterior mass occupying the upper portion of the hemithorax was visualized. The mass was soft, gray-white, and pleural-based. There were 3 smaller satellite lesions occupying the left first, fifth, and eighth intercostal spaces. The tumors peeled readily off the extrapleural plane, and there was no evidence of mediastinal extension or invasion of any structure, including the lung.

Several fragments of tissue were received in pathology, ranging from 9 × 6 × 3.2 cm to 3 × 2 × 2 cm. The specimens were obtained from the largest (posterior mass) and from 2 of 3 smaller satellite lesions. The largest fragment was partly encapsulated, soft, lobulated, and tan. The cut surface showed nodular tan areas surrounded by gray-white smooth areas with areas of cyst formation.

Microscopic examination of all 3 sites was identical and revealed a mass composed of solid areas alternating with less solid areas. In the solid areas, dense accumulations of glomus cells and blood vessels were seen. In the less dense areas, blood vessels surrounded by glomus cells were separated by hyaline stroma. The glomus cells displayed round nuclei with finely dispersed chromatin, eosinophilic cytoplasm, and indistinct cell boundaries. No mitotic activity, nuclear atypia, or necrosis was noted. The cells stained positively with antibodies to actin but not to desmin or factor VIII.

Smooth muscle fibers were seen as large bundles and focally were intimately associated with the glomus cells (Figure). This feature confirmed the diagnosis of a glomangiomyoma variant of glomus tumor.

Glomus tumors have been divided into 3 groups (glomus tumor proper, glomangioma, and glomangiomyoma), depending on the varying proportions of glomus cells, vascular structures, and smooth muscle tissue present. Glomangiomyomas represent less than 10% of glomus tumors and display a gradual transition from glomus cells to elongated, mature smooth muscle cells. This transition is most obvious in the region of large vessels, where the peripheral smooth muscle cells seem to blend with the tumor.1 

Nearly all glomus tumors label positively with antibodies against vimentin and muscle actin isoforms. Desmin immunopositivity is much more variable, however, and contradictory results have been reported in different series.1 

The literature contains 7 previously reported cases of multiple glomangiomyomas, as summarized in the Table. The patients ranged in age from 13 to 62 years and demonstrated a slight male predominance. The extremities, especially the arms, were most often involved by these lesions.

The location of this patient's tumor in the chest wall might be explained by the development of the tumor from an aberrant glomus body at this site. Glomus tumor of the chest wall has been described by Enziger and Weiss.1 

The manner by which glomus tumors become multifocal is a subject of speculation. The multiple gastric glomus tumors proper reported by Haque et al2 demonstrated vascular involvement, and these authors favored this as the explanation for how the tumors in their case became multifocal. Since glomus cells share many features of smooth muscle cells, intravascular spread of glomus tumors may be similar to that seen in intravascular leiomyomatosis. Intravascular tumor has also been described by Michal3 in a case of solitary glomus tumor proper of the foot.

In summary, the multiple glomus tumors in the chest wall of this young man are best categorized as glomangiomyomas, owing to the presence of prominent smooth muscle bundles admixed with glomus cells. Such cases of multiple glomangiomyomas are rare, and to my knowledge have not been described before in the chest wall.