1/94. Pilomatrix carcinoma of the eyelid. PURPOSE: To report a case of a recurring mass with an unusual origin on the eyelid of a 34-year-old man. METHOD: Case report. RESULT: histology demonstrated that the mass was a pilomatrix carcinoma. CONCLUSION: An atypical mass with unusual symptoms or signs needs definitive treatment and diagnostic confirmation with histology. ( info) |
| The association of pilomatrixoma and myotonic dystrophy has been described in the past in 13 publications in the English literature. The association seems to involve the development of pilomatrixomata before signs of myotonic dystrophy. myotonic dystrophy is the commonest adult dystrophy and is an autosomaldominant disease with a variable phenotypic penetrance. The disease is determined by a genetic locus on chromosome 19q and can be diagnosed using methods of dna testing. We describe the 25th case of a patient with both conditions together with a review of the literature. To our knowledge, no other patient has had such a large number of histologically proven pilomatrixomata. ( info) |
3/94. Melanocytic matricoma: a report of two cases of a new entity. Many reports exist of pigmented adnexal tumors containing dendritic melanocytes such as pigmented basal cell carcinomas and pigmented pilomatricomas. Correspondingly, melanocytes are a known component of the bulbs of anagen follicles. The phenomenon of melanization of adnexal tumors highlights the interrelationship between melanocytes and adnexal epithelium and may represent normal melanocytes colonizing a neoplastic proliferation. We report on two cases of a unique tumor composed of neoplastic matrical cells with a significant component of melanocytes. Both cases presented as pigmented papules in older men (66 and 80 years, forearm and pectoral region, respectively). Histologically, these were well-defined nodular proliferations composed of variably melanized, pleomorphic, and mitotically active matrical and supramatrical cells forming clusters of "shadow cells." Admixed with the epithelial cells were numerous melanized dendritic melanocytes. Shadow cells expressed keratin 13, and a subpopulation of S-100 protein-positive dendritic cells were evident. No recurrence of any type was found after reexcisions 4 months and 2 years later. We propose the name of melanocytic matricoma for these two heretofore unreported cases of a unique neoplasm composed of matrical cells and melanocytes recapitulating epithelial-melanocyte interaction in the follicular anagen bulb. Although their small size, circumscription and clinical course suggest a benign nature, melanocytic matricomas' cytologic atypia disclose the potential for malignant behavior. ( info) |
4/94. Pilomatrix carcinoma with multiple metastases: report of a case and review of the literature. 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. ( info) |
5/94. Surgical excision of pilomatrixoma of the head and neck: a retrospective review of 26 cases. The objective of this article is to describe our experiences in treating patients for head and neck pilomatrixoma at our institution during a 5-year period and to compare our findings with previously published results. To that end, we conducted a 5-year retrospective chart review to identify those patients who had a confirmed histopathologic diagnosis of pilomatrixoma involving the head and neck area. We reviewed medical records for presenting signs and symptoms, lesion characteristics, treatment rendered, and outcomes. We identified 26 patients, aged 6 to 77 (mean: 33), who met the criteria for inclusion in our study. All had been treated for solitary tumors with simple surgical excision and closure. We found no reported adverse outcomes and no tumor recurrences at the surgical sites. These findings support the use of simple surgical excision as the treatment of choice for these tumors. ( info) |
6/94. Pilomatricoma associated with several hair follicles. We report two cases of pilomatricoma in which the neoplasm was connected with several hair follicles. A 17-year-old boy developed an erythematous nodule, 1 cm in diameter, in the right temporal region, histologically showing basophilic cells connected with at least two hair follicles. In the other case, a 20-year-old man had a centrally ulcerated tumor, 2 cm in diameter, in the left lower back. Basophilic cells proliferated in connection with at least six hair follicle-like structures, accompanied by the development of shadow cells underneath. The histopathologic findings in these two cases suggest the existence of a subset of pilomatricomas that involve several pre-existing hair follicles. ( info) |
7/94. Unusual multiple pilomatrixomata: case report and review of the literature. Pilomatrixomata are rare, benign, calcifying cutaneous tumours of the hair matrix cells. They usually present in childhood with a pre-surgical diagnosis of sebaceous cyst, because of their size and appearance. The usual sites of occurrence are the scalp, head and neck. Treatment is usually surgical excision. We report a case of unusual, multiple, benign pilomatrixomata in an Indian adult. The plastic surgeon should be aware of the various guises of pilomatrixoma and be alert to the important, although rare, occurrence of malignancy. ( info) |
| Multiple and familial cases of pilomatrixomas have been reported in myotonic dystrophy. We report 2 cases of pilomatrixoma in Turner's syndrome. Between 5 and 19 years of age, 1 patient developed 6 pilomatrixomas including atypical occurrences on the chest and the posterior aspect of the leg. This is the first report of multiple pilomatrixomas in Turner's syndrome. copyright (R) 2000 S.Karger AG, Basel ( info) |
9/94. Atypical adult non-calcified pilomatricoma. We present a rare case of a non-calcified pilomatricoma in a 67-year-old man. This tumor was extremely large in size, and its location, in the lower extremity, was very unusual. The clinical, radiographic, and histopathological features are described in detail. The role of magnetic resonance imaging (MRI) in the diagnosis of this entity is discussed. Definite internal reticulations and septations were observed. A possible explanation for this observation is that the high signal intensity reticulations may represent edematous stroma surrounding basaloid cells. ( info) |
10/94. Giant pilomatricoma simulating carcinoma of the male breast. We present the case of a 65-year-old man with a large calcifying epithelioma in the right breast. The case is unusual not only for the size of the tumour but also for its location in a male breast. Despite its large size (12 x 10 x 7 cm), the tumour did not show any clinical, radiological or histological evidence of malignancy or aggressive behaviour. One year after surgery there was no recurrence or metastatic disease. The possibility of malignancy, recurrence or metastasis of these large tumours, as well as the necessity of follow-up and investigations, are emphasised. ( info) |