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Multiple cutaneous granular-cell tumors

  • Author(s): Hazan, Carole
  • Fangman, William
  • et al.
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Multiple cutaneous granular-cell tumors
Carole Hazan MD, William Fangman MD
Dermatology Online Journal 13 (1): 4

New York University Department of Dermatology

Abstract

A 54-year-old woman presented with subcutaneous nodules and a prior diagnosis of multiple, cutaneous granular-cell tumors. The first case of granular cell tumor was reported in 1854. It remains a rare tumor, which is most commonly seen in women. One-half are usually located in the head and neck region, mostly on the tongue. Approximately one third are cutaneous and 25 percent of the cases occur as multiple lesions.



Clinical synopsis

A 54-year-old woman presented to the Dermatology Clinic at Bellevue Hospital simple excision and without recurrence since March 2002. About 6 months before, the patient noticed three new asymptomatic, subcutaneous masses that were gradually increasing in size. She requested to have these removed. An excision of the lesion on her left forearm was performed and well tolerated.

On her right shoulder, left forearm, and left lateral aspect of the chest were three, soft, flesh-colored, subcutaneous nodules. There was no evidence of any surface change.


Figure 1Figure 2

Figure 3

Histopathology of the dermis reveals plexiform nodules that consist of large, polygonal cells, which have abundant, finely granular, eosinophilic cytoplasm with small, round, centrally located nuclei. The intracytoplasmic granules stain positively with periodic acid-Schiff stain. The cells show a positive immunostain for S100 protein.


Comment

Weber was the first to report a case of granular-cell tumor in 1854. The Schwann-cell origin of granular-cell tumors was reported in the 1960's [1].

Granular-cell tumors are most commonly found in women in the second to sixth decades. They are most commonly seen in African Americans [2]. One-half of the tumors are found in the head-and-neck region, with the tongue being the most common site. One third will develop on the tongue, one third on the skin, and one third in internal organs. In up to 25 percent of individuals, there are multiple lesions [3]. Approximately 1-2 percent of granular-cell tumors are malignant. Features suggestive of malignancy include a size greater than 4 cm, necrosis, metastases to lymph nodes, aggressive clinical behavior and rapid growth, nuclear pleomorphism, and ulceration [4].

Clinical manifestations of granular-cell tumors are varied. Many present as subcutaneous nodules, the overlying skin of which may be normal, hyperpigmented, or with a covering of hair. They can also be either pruritic or painful.

Histopathologic examination shows broad fascicles of tumor cells that infiltrate the dermis among collagen bundles and are arranged in nests or sheets with overlying pseudoepitheliomatous hyperplasia. Small, uniform, eosinophilic granules fill the cytoplasm. The nuclei are small, round-to-oval, and are centrally located. There is often neurotrophic spread along the peripheral nerves. Immunostains are generally positive for S-100 protein, peripheral nerve myelin proteins, and neuron specific enolase [4].

Although, intralesional glucocorticoids have been shown to cause regression in a selected number of cases, a wide local excision is the treatment of choice because they can, although rarely, recur after simple excision.

References

1. Gross VL, Lynfield V. Multiple cutaneous granular cell tumors: a case report and review of the literature. Cutis. 2002;69:343

2. de Misa RF, et al. Pruritic vulvar nodule in a black woman. Diagnosis: granular - cell tumor (Abrikossoff tumor). Arch Dermatol. 2000;136:1165

3. Singh B. Multicentric granular cell tumors. South Med J. 1993;86:1446

4. Schoedel KE, et al. An S100 negative granular cell tumor with malignant potential: report of a case. J Am Acad Dermatol. 1998;39:894

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