Original Paper
Pilomatrix carcinoma with multiple metastases: report of a case and review of the literature

https://doi.org/10.1016/S0959-8049(98)00299-8Get rights and content

Abstract

Pilomatrix carcinoma, the malignant counterpart of pilomatrixoma, is rare, with only 55 cases reported, and only four cases with visceral metastases described in the literature. Here we present a case report and a literature review on this rare tumour. A 74-year-old male with a pilomatrix carcinoma from the left temporal region presented in July 1996 and the tumour was excised. One month after diagnosis, metastases to both lungs and to a regional lymph node were found and histologically verified. The patient also developed metastases in the abdomen, back and thoracic spine. The latter resulted in spinal cord compression and paraplegia. Despite systemic chemotherapy with intravenous cisplatin and 5-fluorouracil and localised radiotherapy to the thoracic spine, progression and deterioration led to death within 3 months from time of diagnosis. Pilomatrix carcinomas are usually indolent. In our patient, however, the malignant disease progressed rapidly and it appeared to be resistant to both chemotherapy and irradiation.

Introduction

Pilomatrixoma was first described in 1880 by Malherbe and Chenantais[1] as a ‘calcifying epithelioma’, initially thought to be derived from sebaceous glands. In 1949, Lever and Griesemer[2] suggested that the origin of the tumour was hair matrix cells. Later, light and electron microscopic observations and histochemical studies have supported this view3, 4, 5, 6 and the name ‘pilomatrixoma’, first proposed by Forbis and Helwig[7] in 1961, has been generally accepted. Pilomatrixomas are slow-growing, benign dermal tumours with possible extension to the subcutaneous (s.c.) tissue[8].

Although the locally aggressive behaviour of some cases of pilomatrixoma was first suggested by Gromiko in 1927[9], for decades it was considered a neoplasm in which malignant transformation did not occur[3]. The malignant variant of pilomatrixoma was not seriously considered until 1980, when Lopansri and Mihm[10] reported a case of aggressive pilomatrixoma and reviewed five similar cases from the literature. They proposed the term ‘pilomatrix carcinoma’ or ‘calcifying epitheliocarcinoma of Malherbe’. Pilomatrix carcinoma is rare and hitherto 55 cases have been reported11, 12, 13, 14, 15, 16, 17, 18.

Recurrences of pilomatrix carcinomas are common, but the metastatic potential has not been acknowledged by all investigators dealing with this malignancy19, 20. Metastasising pilomatrix carcinoma is exceedingly rare, with only four reported cases in the literature12, 21, 22, 23.

We present a case of pilomatrix carcinoma in a 74-year-old male in whom metastases to the neck, both lungs, thoracic spine and possibly the abdomen developed within a few months. The cancer showed an aggressive course with primary resistance to both chemotherapy and radiotherapy.

Section snippets

History

A 74-year-old male presented in July 1996 with a tumour in the left temporal region, which had been first noticed 2 months earlier. The patient had a previous history of occasional alcohol abuse and a lumbar disc herniation in 1977, but was otherwise healthy. The tumour, 4×3 cm and elevated 1 cm above the skin surface, was excised mid July. Skin was transplanted from the left upper arm to cover the defect. The histological diagnosis was pilomatrix carcinoma with non-radical surgical margins. For

Discussion

Although Gromiko even in 1927[9] noted the aggressive behaviour of some pilomatrixomas, this tumour was considered to be benign without any malignant potential[3]. Surgical reports24, 25, 26, during the 1970s, of locally aggressive pilomatrixomas led to the introduction of the terms ‘pilomatrix carcinoma’ and ‘calcifying epitheliocarcinoma’ by Lopansri and Mihm[10] in 1980. Their malignant terms were based on a review of five cases of ‘pilomatrixoma with aggressive behaviour’ and a case of

Acknowledgements

The financial support from the Norwegian Cancer Society is greatly appreciated.

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