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• W2327984985 abstract "Sir: On close review of the February 2014 Hand/Peripheral Nerve article entitled “Hand Tumors: I. Skin and Soft-Tissue Tumors of the Hand,”1 our group noticed that glomulovenous malformations, a rare and often overlooked entity in the plastic surgery literature, were not discussed. The following is a brief overview of these lesions and a case of multiple upper extremity glomulovenous malformations. Described by Masson in 1924, glomus tumors are rare benign vascular lesions arising from the glomus body, an arteriovenous anastomosis that thermoregulates acral skin.2,3 They constitute 1 to 2 percent of soft-tissue tumors. Glomus cells have a monomorphic, basaloid shape, with short interdigitating cytoplasmic processes containing actin filament bundles and outer membrane plaques that tether tightly to subcutaneous tissue. Two histologic variants of glomus tumors are defined by their relative abundance of glomus cells and vascular architecture2–4: Solid glomus tumor (90 percent): encapsulated glomus cell bundles surrounding small vascular lumina. Glomulovenous malformation (10 percent): prominent dilated, cavernous channels lined by endothelium with underlying glomus cells. Of note, glomulovenous malformations can be genetic (autosomal dominant inheritance), resulting from loss-of-function mutation to the glomulin allele (1p21), which regulates smooth-muscle cell differentiation.5 Glomus tumor subtypes vary in demographic and anatomical distribution. Solid glomus tumors are most common, typically presenting in adulthood as solitary, firm, subungual lesions. They are typically smaller than 1 cm and unanimously painful in response to touch or temperature change; therefore, pain can be provoked by applying ice to the lesion in question.2,3,6 In contrast, glomulovenous malformations are most often congenital, presenting in childhood as multiple nodules restricted to the deep dermis of the upper extremities or trunk. Adult glomulovenous malformations are rare, constituting less than 10 percent of total glomus tumor cases. Glomulovenous malformations can be further distinguished from solid glomus tumors in that they tend to be larger, and only 42 to 63 percent of patients report pain at presentation.6 We treated a 44-year-old man who presented to our clinic with multiple nodules smaller than 2 cm on bilateral forearms and wrists. The lesions had been steadily increasing in size and pain over 2 years. His father had similar upper extremity nodules treated surgically at age 30 (pathologic condition unknown). Our patient’s lesions were treated with simple surgical excision. Intraoperatively, they had a bright red hue and spongy consistency (Fig. 1). They were tightly tethered to the deep dermis and forearm muscle fascia. No obvious efferent or afferent vessels were noted.Fig. 1: Dissection of glomulovenous malformation (left wrist), tethered to subcutaneous tissues without any apparent vascular inflow or outflow.Final pathologic findings were consistent with glomulovenous malformations (Fig. 2). At 5 months postoperatively, the patient is without recurrence.Fig. 2: (Above) Photomicrograph showing a somewhat circumscribed cavernous hemangioma-like nodule, with perivascular glomus cell proliferation (original magnification, ×40). (Below) Photomicrograph highlighting typical morphologic appearance of glomus cells with centrally placed round, regular nuclei with an abundant amphophilic to eosinophilic cytoplasm clustered around thick-walled dilated vessels (original magnification, × 200).The definitive treatment for symptomatic glomulovenous malformations is simple excision.3–6 However, small glomulovenous malformations, once confirmed by tissue biopsy, may be amenable to laser ablation or sclerotherapy.4 If lesions violate fascia and infiltrate deep into muscle, malignant degeneration should be considered and surgical resection with clear margins is imperative. Thus, we argue that glomulovenous malformations are an important consideration in the differential diagnosis of multiple subcutaneous lesions of the adult upper extremity. ACKNOWLEDGMENT The authors are grateful to Yao-Tseng Chen, M.D., Pathology Department, New York-Presbyterian Hospital, for providing glomulovenous malformation specimens. DISCLOSURE The authors have no financial interest in any of the products or devices mentioned in this communication. No outside funding was received. Justin L. Perez, B.S. Weill Cornell Medical College Leslie Cohen, M.D. Thomas A. Imahiyerobo, M.D. Division of Plastic Surgery Paula S. Ginter, M.D. Department of Pathology David M. Otterburn, M.D. Division of Plastic Surgery Weill Cornell Medical College New York, N.Y." @default.
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• W2327984985 date "2014-12-01" @default.
• W2327984985 modified "2023-09-26" @default.
• W2327984985 title "Let’s Keep Our Differential Broad" @default.
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• W2327984985 doi "https://doi.org/10.1097/prs.0000000000000714" @default.
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