Granular cell tumors display malignant behavior

"It was unusual that this woman continued having recurrent tumors. If you look in the literature, the definitions of malignancy don't apply to her case. Yet she's had a traumatic experience, including a tracheostomy, because of this." says Catherine D. Carretero, M.D., senior resident, Henry Ford Hospital, who treated the patient in conjunction with Earl Rudner, M.D., also of Henry Ford Hospital.

"She didn't have cytologic atypia, rapid growth or metastases, but she did continue to develop multiple recurrent lesions even after excision. Typically, when one excises the tumor it doesn't return. Literature review points out that even when one excises it and the margins are positive, these tumors still usually do not recur (Gross VL, Lynfield Y. Cutis. 2002 May;69(5):343-346., Curtis BV, Calcaterra TC, Coulson WF. Head Neck. 1997 Oct;19(7):634-637.)," she adds.

Presentation When the patient arrived at Henry Ford Hospital, she presented with an asymptomatic firm nodule of the tongue that she'd had for five years. Upon examination, physicians noted a 1 cm, firm, flesh-colored nodule on her right dorsal tongue.

The nodule caused no overlying epidermal change. On previous biopsy from the University of Iowa, doctors observed that granular cells had invaded the muscle tissue surrounding the nodule, which commonly occurs with this type of tumor. The nodule itself was comprised of a dense layer of collagen infiltrated by cells with a distinct membrane, along with pale, faintly eosinophilic granule-filled cytoplasm and round to oval-shaped nuclei.

While staining was not performed on this specimen, granular tumors typically stain positive for S-100 protein, peripheral nerve myelin proteins and neuron-specific enolase. The cytoplasmic granules, on the other hand, stain positive for CD68. The patient's lab results, including CBC, chem 7 and LFTs, remained within normal limits. The patient also was ANA, SSA, SSB and RF negative.

Excision referral Dr. Carretero and her colleagues referred the patient for excision and have not seen her since September 2004. However, the patient has been followed more closely by Henry Ford Hospital's pulmonary department.

"She continues having recurrent tumors in the lungs as well which are affecting her breathing," Dr. Carretero says. "It's been a problem because it has been impossible to resect the pulmonary lesions due to the surrounding anatomy. The lesions in the lungs have caused a significant morbidity. Her breathing difficulty is very serious" and potentially fatal should the condition continue to worsen.

Multiple lesions Up to 25 percent of patients with granular cell tumors have multiple lesions. In 1 percent or 2 percent of cases, these lesions will spread in a fashion that appears malignant, at least on first glance. Wide local excision remains the treatment of choice for granular cell tumors. When these tumors measure more than 4 cm or grow rapidly, doctors typically recommend regional lymph node dissection.

Dr. Carretero says, "We don't see granular tumors on a daily basis. And when we do, they're usually a solitary tumor. But dermatologists should recognize there could be multiple lesions that can cause significant morbidity. If the patient is having other symptomatology that doesn't seem related to the tumor one might see on the tongue, it would be worth doing an investigation because the lesions can be multiple."

Disclosure: Dr. Carretero reports no financial interests concerning this article.

For more information: http://www.henryfordhealth.org/