Multiple Morphologically Distinct Cutaneous Granular Cell Tumors Occurring in a Single Patient
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Multiple Morphologically Distinct Cutaneous Granular Cell Tumors Occurring in a Single Patient Livia Van, MD; Sareeta R.S. Parker, MD PRACTICE POINTS • Granular cell tumors (GCTs) typically present as solitary lesions; however, multiple lesions occur in approximately 25% of cases. • Granular cell tumors have a variable clinical appearance and may mimic malignant neoplasms (eg, squamous cell carcinoma) as well as infectious diseases (eg, condyloma, syphilis). • The histological features of GCTs are distinctive, including an unencapsulatedcopy dermal proliferation of monotonous polygonal cells with indistinct borders and fine, granular, eosinophilic cytoplasm arranged in irregular sheets and nests. not Do Granular cell tumors (GCTs) typically are benign Histopathology of several lesions was diagnostic solitary tumors derived from Schwann cells. The of GCTs. This case illustrates the highly varied tongue and skin are the most common sites of clinical presentation and morphology of cuta- involvement; however, lesions also can develop neous GCTs, even those occurring in a single in viscera such as the gastrointestinal tract. Mul- patient. In addition to mimicking other benign tiple cutaneous GCTs in a single patient have neoplasms, GCTs may mimic other disease pro- been reported, with the lesions being described cesses, including malignant lesions, infections, as subcutaneous papules,CUTIS nodules, or verrucous and inflammatory disorders. Skin biopsy generally nodules. We report the case of a patient who is required for definitive diagnosis. presented with several simultaneously occurring Cutis. 2016;97:E26-E29. cutaneous GCTs with morphologically distinct clinical appearances ranging from subcutaneous Case Report nodules with no overlying epidermal alteration to A 27-year-old black man was admitted to the exophytic moist nodules with eroded surfaces. hospital with chills; night sweats; unintentional 25-lb weight loss; and multiple widespread, painful, progressively enlarging skin nodules of 3 months’ duration. The lesions had first developed on the back Drs. Van and Parker were from the Department of Dermatology, and later appeared on the face, trunk, arms, thighs, Emory University School of Medicine, Atlanta, Georgia. Dr. Parker and genital region. He denied dysuria or urethral currently is from the Department of Dermatology, Kaiser Permanente discharge. He had a remote history of adequately Southwood Specialty Center, Jonesboro, Georgia, and Grady Health treated chlamydia infection but no other remarkable System, Atlanta. personal or family history. The authors report no conflict of interest. Correspondence: Sareeta R.S. Parker, MD, Dermatology, Kaiser Physical examination revealed a thin man with Permanente Southwood Specialty Center, 2470 Mt Zion Pkwy, more than 20 lesions on the face, trunk, arms, Jonesboro, GA 30326 ([email protected]). thighs, and genital region ranging in size from E26 CUTIS® WWW.CUTIS.COM Copyright Cutis 2016. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Granular Cell Tumors 1 to 4 cm. Lesion morphologies varied greatly and venereum (LGV) serum antibodies also were posi- included subcutaneous firm nodules with no epider- tive. Human immunodeficiency virus screening was mal change (Figure 1); dermatofibromalike nodules negative. Positron emission tomography–computerized with overlying erythema and hyperpigmentation tomography revealed enlarged and hypermetabolic (Figure 2); condylomalike, verrucous, pink papulo- lymphadenopathy above and below the diaphragm. nodules (Figure 3); ulcerated angular plaques with After therapy with intravenous penicillin G and rolled borders and palpable tumor extension deep oral doxycycline for concurrent secondary syphilis (1–2 cm) to the subcutis (Figure 4); and a vegeta- and LGV, the patient’s macular eruption and consti- tive, eroded, exophytic tumor with palpable deep tutional symptoms resolved within weeks of the ini- extension (Figure 4). A diffuse, erythematous, macu- tial presentation. His lymphadenopathy improved, lar eruption also was noted on the trunk and bilat- his rapid plasma reagin titer decreased, and his eral arms and legs including the soles of both feet chlamydia RNA became undetectable. However, the along with nontender cervical, axillary, and inguinal skin lesions remained unchanged. lymphadenopathy. The ocular, oral, and nasal muco- sae were not affected. The differential diagnosis for each lesion differed based on morphology. Infectious, inflam- matory, and neoplastic processes were considered, including syphilis, dermatofibroma, dermatofibro- sarcoma protuberans, metastatic disease, leukemia cutis, sarcoidosis, panniculitis, condyloma acumina- tum, and vegetative herpes simplex virus infection copy (inguinal lesion). Laboratory data revealed a reactive rapid plasma reagin with treponemal IgG titers of 1:64. Urine chlamydia RNA probe and lymphogranuloma not Do Figure 2. Firm dermal papule on the anterior aspect of the left shoulder with violaceous hyperpigmenta- CUTIS tion (dermatofibromalike). Figure 1. Firm subcutaneous nodules on the back with Figure 3. Verrucous pink papule on the right side of no epidermal change. the neck. WWW.CUTIS.COM VOLUME 97, APRIL 2016 E27 Copyright Cutis 2016. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Granular Cell Tumors Incisional biopsies of 4 clinically distinct skin All 4 lesions had histopathologic findings charac- lesions revealed well-delineated dermal prolifer- teristic of granular cell tumors (GCTs). A lesion in ations of cells with eosinophilic granular cyto- the left inguinal region (Figure 4 [medial lesion]), plasm and indistinct cell borders (Figure 5). which initially was thought to be condyloma latum Two specimens displayed marked epidermal hyper- or a squamous cell carcinoma (SCC), also was later plasia (Figure 6). No atypical mitotic figures were confirmed to be a GCT. identified. Immunohistochemistry for S-100 pro- Repeat positron emission tomography– tein was diffusely positive in the neoplastic cells. computerized tomography several weeks later Immunohistochemistry for Treponema pallidum was confirmed resolution of the previously noted negative. No mycobacterial or fungal organisms were lymphadenopathy. Although 2 GCTs have not identified in acid-fast bacillus, periodic acid–Schiff, recurred after biopsy, the other 2, which the patient or Gomori methenamine-silver–stained sections. refused to have completely excised, continued to grow. Follow-up 2.5 years after hospitalization revealed persistence of the lesions with no remark- able morphological changes. Comment First described in 1854, GCTs are uncommon neo- plasms of probable Schwann cell origin that can arise in almost any location of the body but most often appear on the skin and in the subcutaneous tissues and oralcopy cavity.1,2 The commonly regarded rule of thirds describes its most favored locations: one-third on the tongue, one-third on the skin, and one-third in internal organs.3,4 Granular cell tumors occurnot with greater frequency in adults, females, and black individuals.1-5 Cutaneous GCTs usually present as solitary asymptomatic masses; however, multiple tumors Dohave been noted in up to 25% of reported cases.4,6 Figure 4. Ulcerated angular plaque in the left inguinal/ In children, multiple cutaneous GCTs have been genital area with rolled borders and tumor extension reported in the setting of neurofibromatosis type I as deep to the subcutis adjacent to a vegetative, eroded, 2,5,7-9 exophytic tumor with palpable deep extension. well as with other disorders. CUTIS dvn kdvlkv Figure 5. Large polygonal cells with eosinophilic granu- lar cytoplasm, prominent bland nuclei, and indistinct cell Figure 6. Marked pseudoepitheliomatous hyperplasia borders (H&E, original magnification ×40). (H&E, original magnification ×10). E28 CUTIS® WWW.CUTIS.COM Copyright Cutis 2016. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Granular Cell Tumors Cutaneous GCTs have been reported to range nodes were observed.4,5 Histologically, nuclear pleo- from sessile, pedunculated, or verrucous nodules to morphism and atypia, cell spindling, vesicular nuclei subcutaneous papules and nodules with no epidermal with prominent nucleoli, necrosis, and high mitotic change. Our case not only illustrated the diverse activity favor malignancy.1,3 clinical appearance of cutaneous GCTs but also dem- Treatment is complete surgical excision. onstrated multiple morphologically distinct cutane- Observation is acceptable if tumors are asymptom- ous GCTs occurring in a single patient. Of particular atic and do not impede function. Regression of some interest is our patient’s coexisting secondary syphilis GCTs has been induced with use of intralesional and LGV infections, which can pose a diagnostic corticosteroids.5 Spontaneous regression is rare. dilemma to the unsuspecting clinician. The manifold Prior reports have emphasized the importance of appearances of this patient’s GCTs resulted in a broad long-term follow-up in patients with multiple GCTs differential diagnosis. Syphilis (condyloma latum), to monitor for development of systemic lesions.4 condyloma acuminatum, LGV, metastatic disease, Kaposi sarcoma, lymphoma, dermatofibrosarcoma REFERENCES protuberans, leiomyoma, SCC, and deep fungal and 1. Apisarnthanarax P. Granular cell tumor.