Final Diagnosis -- A 62 year-old man with glomus tumor of the kidney

FINAL DIAGNOSIS    GLOMUS TUMOR OF THE KIDNEY

DISCUSSION

Glomus tumors arise from the glomus body, a specialized smooth muscle-derived structure that regulates blood flow for thermal regulation [1]. Approximately 10 percent of glomus tumors are multiple, and in some instances, they are familial [2]. Most patients with glomus tumors are young to middle aged adults [1]. These lesions typically occur as painful skin nodules in the upper extremities, most often the subungual region of the fingers, followed by other portions of the distal extremities including the wrist, palm, and foot as the most common sites[1]. Internal locations such as mediastinum, lung, trachea, and stomach are unusual for involvement by glomus tumor [3, 4]. Glomus tumor arising within the kidney is exceptionally rare, with six cases described [5].

The histologic differential diagnosis of glomus tumor of the kidney includes other neoplasms with myoid or pericytic differentiation (hemangiopericytoma, juxtaglomerular cell tumor, leiomyoma, and angiomyolipoma), neuroendocrine tumors (carcinoid tumor and paraganglioma) and metanephric adenoma. Histologic features and a panel of immunohistochemical stains differentiates glomus tumor from these entities.

Glomus tumor can have a hemangiopericytoma-like vascular pattern. However, hemangiopericytomas are smooth muscle actin negative, unlike glomus tumor [6]. Juxtaglomerular cell tumor presents in a much younger population than glomus tumor and virtually all patients are hypertensive due to the secretion of renin by tumor cells [7, 8]. Immunohistochemically, juxtaglomerular cell tumor can be separated from glomus tumor by strong renin and CD34 positivity and focal patchy staining of smooth muscle actin [8]. Ultrastructurally, renin-specific granules are present.8 Angiomyolipoma, with a predominant muscular component might enter the differential diagnosis but can be separated from glomus tumor by HMB-45 or melan-A positivity of the perivascular epithelioid cells [9]. Although glomus tumors in which the cells form nests might be confused with paraganglioma, lack of chromogranin and synaptophysin expression clarifies the diagnosis. Leiomyomas, particularly the epithelioid variant can be a part of the differential diagnosis, but they are usually strongly desmin positive. Metanephric adenoma can be differentiated from glomus tumor by positive WT1 and S100 staining [10-12].

In conclusion, this lesion is difficult to distinguish from other renal neoplasms by radiographic imaging. Histologic and immunohistochemical overlap with other entities that occur in the kidney can lead to an incorrect diagnosis. Careful histologic examination and immunohistochemical interpretation are necessary to make the diagnosis of glomus tumor of the kidney, which shows an excellent clinical outcome.

REFERENCES

  1. Carroll RE, Berman AT. Glomus tumors of the hand: review of the literature and report on twenty-eight cases. J Bone Joint Surg Am. 1972;54:691-703.
  2. Calduch L, Monteagudo C, Martinez-Ruiz E, Ramon D, Pinazo I, Carda C, Jorda E. Familial generalized multiple glomangiomyoma: report of a new family, with immunohistochemical and ultrastructural studies and review of the literature. Pediatr Dermatol. 2002;19:402-408.
  3. Gaertner EM, Steinberg DM, Huber M, Hayashi T, Tsuda N, Askin FB, Bell SW, Nguyen B, Colby TV, Nishimura SL, Miettinen M, Travis WD. Pulmonary and mediastinal glomus tumors-report of five cases including a pulmonary glomangiosarcoma: a clinicopathologic study with literature review. Am J Surg Pathol. 2000;24:1105-1114.
  4. Miettinen M, Paal E, Lasota J, Sobin LH. Gastrointestinal glomus tumors: a clinicopathologic, immunohistochemical, and molecular genetic study of 32 cases. Am J Surg Pathol. 2002;26:301-311.
  5. Sasaki K, Bastacky SI, Hrebinko RL, Parwani AV, Zynger DL. Glomus tumor of the kidney: case report and literature review. Int J Surg Pathol. 2009;Jan 28.
  6. Porter PL, Bigler SA, McNutt M, Gown AM. The immunophenotype of hemangiopericytomas and glomus tumors, with special reference to muscle protein expression: an immunohistochemical study and review of the literature. Mod Pathol. 1991;4:46-52.
  7. Martin SA, Mynderse LA, Lager DJ, Cheville JC. Juxtaglomerular cell tumor: a clinicopathologic study of four cases and review of the literature. Am J Clin Pathol. 2001;116:854-863.
  8. Markey RB, MacLennan GT. Juxtaglomerular cell tumor of the kidney. J Urol. 2006;175:730.
  9. Ashfaq R, Weinberg AG, Albores-Saavedra J. Renal angiomyolipomas and HMB-45 reactivity. Cancer. 1993;71:3091-3097.
  10. Fischer EG, Carney JA, Anderson SR, Klatt EC, Lager DJ. An immunophenotypic comparison of metanephric metaplasia of Bowman capsular epithelium with metanephric adenoma, Wilms tumor, and renal development: a case report and review of the literature. Am J Clin Pathol. 2004;121:850-856.
  11. Azabdaftari G, Alroy J, Banner BF, Ucci A, Bhan I, Cheville JC. S100 protein expression distinguishes metanephric adenomas from other renal neoplasms. Pathol Res Pract. 2008;10:719-723.
  12. Muir TE, Cheville JC, Lager DJ. Metanephric adenoma, nephrogenic rests, and Wilms' tumor: a histologic and immunophenotypic comparison. Am J Surg Pathol. 2001;25:1290-1296.

Contributed by Kotaro Sasaki, MD, Sheldon I. Bastacky, MD, Anil V. Parwani, MD. PhD and Debra L. Zynger, MD



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