Pilomatrix carcinoma

Department of Dermatology, Walter Reed Army Medical Center, Washington, DC 20307-5001.
Cancer (Impact Factor: 4.89). 04/1993; 71(8):2491-8. DOI: 10.1002/1097-0142(19930415)71:8<2491::AID-CNCR2820710811>3.0.CO;2-I
Source: PubMed


Pilomatrix carcinoma, a malignant variant of pilomatrixoma, is extremely rare. The authors report 20 patients with pilomatrix carcinoma and review the pertinent literature.
Tumors showing histologic features of pilomatrix carcinoma were selected from the files of the Armed Forces Institute of Pathology. Clinical data of the 20 selected patients were reviewed, and follow-up information was obtained. Sections stained with hematoxylin and eosin were studied in all patients. Special stains were used in selected patients.
Pilomatrix carcinomas were asymptomatic dermal and subcutaneous masses with a predilection for the posterior neck and back. Tumors varied in size, from 1-10 cm (mean, 4.6 cm), and occurred more often in middle-age men, with a male:female ratio of 4:1 (mean age, 45 years). Histologically, pilomatrix carcinomas are characterized by sheets and islands of proliferating atypical basaloid cells with an infiltrating border. Transition to squamous cells, clear cells, areas of necrosis and mitoses often are seen. Keratinization with formation of keratin cysts, shadow cells, and trichohyalin and keratohyalin granules are found in all tumors, in conjunction with calcification and foreign body giant cell reaction, just as are seen in benign pilomatrixoma. Follow-up of 17 patients revealed local recurrence in 10 (59%), with multiple recurrences in 3. One patient had pulmonary metastasis, and one died of extensive local spread of the tumor.
Pilomatrix carcinomas are locally aggressive tumors that have a tendency to recur, especially when they are incompletely excised. Greater anaplasia and deep soft tissue infiltration were associated with a higher incidence of recurrence and death. Wide excision is the preferred treatment. The role of radiation therapy is unclear.

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    • "Pilomatrix carcinoma is a locally aggressive tumor, usually occurring on the head and neck. A literature review revealed that the mean age of diagnosis with pilomatrix carcinoma is 45 years, and it occurs more often in middle-aged males, with a male to female ratio of 4:1 (1). In numerous patients diagnosed with pilomatrix carcinoma, a direct invasion to the adjacent bone has been observed (2,3). "
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    ABSTRACT: Pilomatrix carcinoma is an extremely rare skin tumor derived from basaloid cells in the hair follicles; it often exhibits locally aggressive behavior with a tendency toward local recurrence. The average age of occurrence is 45 years, and there appears to be a male to female incidence ratio of 4:1. Although pilomatrix carcinomas are predominantly identified in the neck and scalp, there are studies in the literature reporting other tumor development sites, including the upper extremities, torso and popliteal fossa. If diagnosed at an early stage, this malignant tumor is generally treated with wide surgical resection. However, for the advanced-stage tumors, there are no standard treatment procedures known to produce good results. The current study presents the case of a 76-year-old male with pilomatrix carcinoma originating from the scalp with metastases to the lung. The patient had a rapid and complete clinical response following an oral combination chemotherapy regimen of cyclophosphamide and etoposide.
    Full-text · Article · Jun 2014 · Oncology letters
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    • "A calcifying epithelioma of Malherbe occurs most commonly in head and neck areas, especially cervical, frontal and temporal regions, eyelids and preauricular regions [5,6]; although the upper extremities, trunk and lower extremities can be affected, in decreasing order of frequency [7]. "
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    ABSTRACT: Although pilomatrixomas are frequently encountered by dermatologists and pathologists in the differential diagnosis of head and neck lesions, this is not usually the case among head and neck surgeons. A pilomatrixoma (calcifying epithelioma of Malherbe) is a benign tumour of the hair matrix cells. Histologically it is characterised by the presence of ghost cells, basophilic cells and foreign body cells. It may sometimes be difficult to histologically distinguish it from its malignant counterpart, the pilomatrix carcinoma. We report an interesting case of an ulcerated pilomatrixoma of the pinna in a middle-aged Caucasian female. A 46-year-old Caucasian female presented with a one-month history of tender brownish lump on the pinna. Initially it was thought to represent a pyogenic granuloma. The lesion was treated by wide circular excision. Histopathological evaluation reported a benign calcifying epithelioma of Malherbe. A search of the world's literature has led us to believe that this is a rare case of a calcifying epithelioma of Malherbe of the pinna. The rapid growth and ulcerative nature of this tumour makes this case even more unique.
    Full-text · Article · May 2012 · Head & Neck Oncology
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    • "These adenoid basal lesions show peripheral palisading, but without any stromal reaction and have no malignant potential [8,9]. In the largest published series of pilomatrix carcinomas to date, Sau [10] et al. have demonstrated that pilomatrix carcinomas have a predilection for males and are commonly located on the head and neck. In their series of twenty patients, at least one died of metastatic disease. "
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    ABSTRACT: Pilomatrix carcinoma is the rare malignant counterpart of pilomatrixoma, a skin adnexal tumour originating from hair matrix cells. Pilomatrix carcinoma can arise as a solitary lesion de novo, or through transformation of a pilomatrixoma. Pilomatrixoma was first described erroneously as being of sebaceous gland origin but was later discovered to be derived from hair matrix cells. They are rare, slow growing tumours of the skin found in the lower dermis and subcutaneous fat and are predominantly found in the neck and the scalp. While known to be locally aggressive, no malignant form was thought to exist until it was described relatively recently. Since then, approximately ninety cases of pilomatrix carcinoma have been reported. We report the case of a 41 year old mentally retarded male who had a longstanding lesion in the left neck for approximately fifteen years previously diagnosed as a pilomatrixoma. He presented with severe headache, falls and visual disturbance and a biopsy showed pilomatrix carcinoma of the occipital region which, on computed tomography ( CT ) invaded the occipital bone, the cerebellum and the left temporal lobe. At his initial presentation he had a craniotomy and subtotal excision of the lesion but received no adjuvant therapy. After an early intracranial recurrence he had further debulking and adjuvant external beam radiotherapy. He has had no further intracranial recurrence after three and a half years of follow-up. Here we present the pathological features of this uncommon tumour.
    Full-text · Article · Dec 2008 · Diagnostic Pathology
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