Ossified neurofibroma

A case of cutaneous neurofibroma that had undergone metaplastic ossification is described.

DEBA P. SARMA, MD* JOHN ROBICHAUX, MDt ALEXANDER FONDAK, MDt New Orleans

e report an unusual case of cutaneous neurofibroma that had undergone metaplastic ossification. Case report
A 61-year-old man had had an asymptomatic mass in his right thigh for 25 years. The lesion had begun as a papule and over the years slowly grew to become a tumorous mass. He denied any trauma to the lesion. The mass, located medially on the patient's right thigh, was a 5 x 3 cm sessile, lobulated, skin-toned tumor. It was very firm and nontender and appeared to be attached to the deep dermis. The regional lymph nodes were not enlarged. His medical history and the remainder of the physical examination were unremarkable. Findings on routine laboratory studies, including measurements of serum calcium and phosphorus levels, were within normal limits. A roentgenogram of the excised specimen showed extensive ossification of the lesion (Fig 1). The bisected lesion showed firm, white nodular mixed with boney fragments. Microscopically (Fig 2), the nodular tissue consisted of thin elongated fibrils with spindle nuclei devoid of atypicality or mitoses. There were areas of myxoid degeneration. Mature compact bone spicules were seen closely mingled with the spindled neural tissue. The tumor was well circumscribed, but not encapsulated. The overlying epidermis was unremarkable. Pathologically the lesion appeared to be neurofibroma with metaplastic ossification.

Fig 1. Roentgenogram of the excised lesion showing extensive ossification.

Comment Cutaneous ossification has been classified as primary when it occurs in the absence of any previous lesion and as secondary when it occurs through metaplasia within a pre-existing lesion. Secondary or metaplastic cutaneous
* Associate Professor of Pathology and Dermatology, Louisiana State University Medical School, and Staff Pathologist, Veterans Administration Medical Center, New Orleans, La. t Chief Resident of Dermatology, Louisiana State University Medi cal School, New Orleans, La. t Assistant Professor of Dermatology, Louisiana State University Medical School, New Orleans, La.

Fig 2. Photomicrograph showing mature bone with the neurofibroma (H&E x 100). June 1983 - Vol. 135, No. 6

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The Journal of the Louisiana State Medical Society.

ossification may be seen in association both with neoplas-tic and with non-neoplastic conditions of the skin.1 In a review of 120 cases of cutaneous ossification and extensive review of the literature, Roth et al2 have noted that the skin tumor most commonly undergoing metaplastic ossification is pilomatrixoma (calcifying epithelioma of Malherbe). Other tumors undergoing ossification are basal cell carcinomas and chondroid syringomas (mixed tumor). Very rarely, ossification has been seen in heman-giomas, chondromas, seborrheic keratoses, trichoepithe-liomas, epidermal cysts, neurilemomas, and intradermal nevi. Roth et al2 noted only one case of neurilemoma out of 425 cases of cutaneous ossification. Our report adds one more case of rare ossification in a neural tumor of the skin. In our case there was no cartilage-like transformation, the spicules of mature bone were within the tumor tissue, and the tumor cells abutted directly on the bone surface, indicating that the neurofibroblasts probably directly transformed into osteocytes, a process similar to membraneous ossification.
References
1. Lever WF, Schaumburg-Lever G: Histopathology of the Skin, ed. 5. Philadelphia, JB Lippincott Co. 1975; pp 626-629. 2. Roth SI, Stowell RE, Helwig EB: Cutaneous ossification. Arch Pathol 1963;76:56-66

June 1983 - Vol. 135, No. 6

Sarma DP, Robichaux J, Fondak A (1983): Ossified neurofibroma. J LA State Med Soc 135:22-23
PMID: 6411839 [PubMed - indexed for MEDLINE]