Pilomatrix carcinoma or calcifying epitheliocarcinoma of malherbe. A case report and review of literature

Abstract

We report a case of aggressive pilomatrixoma with two recurrences. This is the first reported case of such a tumor with a basal cell-like histology of recurrences. We propose that this type of tumor be designated "calcifying epitheliocarcinoma of Malherbe" or "pilomatrixoma carcinoma."Cancer 45:2368-2373, 1980.ILOMATRIXOMA was first described in 1880 by P Malherbe as a benign calcifying tumor believed to arise from the sebaceous gland. It was not until 1961 when Forbis and Helwig reviewed 228 such tumors that the name "pilomatrixoma" was proposed.Evidence by both light and electron microscopic observation and histochemical studies favors the hair matrix as the site of origin. This tumor usually appears as a slow-growing dermal or subcutaneous mass less than 3 cm in size, most commonly located on the head or neck. It is frequently a tumor of the younger age-group with a peak incidence between eight and 13 years. More than 60% of the tumors appear before the third decade. The female to male ratio is nearly 3:2. Familial occurrence has been reported in 7 cases of pilomatrixoma." Gardner's syndrome and myotonic muscular dystrophy have been associated with cases of pilomatrixoma. Those occurring in patients with myotonic dystrophy are often multiple and are commonly familiaL2 Multiple tumors occur in 2-3 S%.ll*ls Recurrences after tumor removal are rare. In the re- view by Forbis and Helwig, 6 patients were described as having recurrences, occurring after periods ranging from one month to six years. Details of the clinical or microscopic appearance of those cases were not discussed. In 1927, Gromiko reported a case of calcifying epithelioma with three recurrences necessitating amputation of the right arm.5 Nonetheless, a malignant counterpart of pilomatrixoma has never been designated with certainty. One tumor was called a "giant calcifying epithelioma," even though it behaved in an aggressive fashion with recurrence^.'^ Hence, we are prompted to report a case wbich we think represents pilomatrix carcinoma. Case ReportA 35-year-old white man presented with an approximate one-year history of a 1.5-cm slowly growing, asymptomatic, movable subcutaneous nodule on the back at the right suprascapular area. The tumor eventually recurred twice and was widely reexcised each time, a t 15 months and 28 months after first therapy. Each excision included the underlying fascia and the superior portion of the subadjacent muscle. The first recurrence presen'ted as three small nodules, the second as a 1-cm dermal and subcutaneous mass. Local radiotherapy was given after the second removal without evidence of clinical recurrence. The patient remains without evidence of recrudescence 40 months after primary therapy. Histpathologic FindingsThe original specimen was a 1.5-cm soft, pink-tan circumscribed dermal and subcutaneous mass containing focal areas of cheesy material. Microscopic examination showed it to be a pseudoencapsulated mass com-

A systematic review of clinical outcomes for patients diagnosed with skin cancer spinal metastases

“…39 Lopansri and Mihm in 1980 proposed the hair matrix as the site of origin. 63 Pilomatrix carcinoma commonly presents in the head or neck region as a slow-growing dermal or subcutaneous mass less than 3 cm in diameter. The peak incidence is estimated to occur between 8 and 13 years of age with tumors being identified before the 3rd decade of life in more than 60% of cases.…”

“…The peak incidence is estimated to occur between 8 and 13 years of age with tumors being identified before the 3rd decade of life in more than 60% of cases. 11,63 Pilomatrix carcinoma shows a predilection for females and is associated with various clinical syndromes. 63 The exact rate of metastases is unknown and reports estimate that the metastases probability is approximately 12% with rare bone metastases.…”

“…11,63 Pilomatrix carcinoma shows a predilection for females and is associated with various clinical syndromes. 63 The exact rate of metastases is unknown and reports estimate that the metastases probability is approximately 12% with rare bone metastases. 11 Pilomatrix carcinoma is poorly responsive to chemoradiation, and is most often treated with wide surgical excision.…”

Gaint Pilomatrixoma on Chest wallA Rare Case Report with Review of Literature

“…it is very rare presentation to see pilomatrixoma in a male patient and that too on chest wall. In 1980 Lapansri et al described about malignant pilomatrixoma which is rare and only a few articles are available 4 . It is an aggressive neoplasm which has high chances of recurrence .…”

Malignant adnexal neoplasms

“…In contrast, pilomatrix carcinoma shows broad geographic necrosis with an infiltrative margin and cytological alterations, including fully transformed malignant change. 90,91,[103][104][105][106][107][108][109][110][111][112][113][114][115] Matrical differentiation may only be focal in these lesions. Tumors have the potential to spread to regional lymph nodes and produce fatal disseminated metastatic disease.…”