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1/9. Two cases of multiple giant pilomatricoma.

    Pilomatricoma is a skin appendage tumor manifestating as a firm, solitary lesion of the face and upper extremity. It generally measures 0.5 to 3.0 cm in diameter and is typically found in young people. We report a 29-year-old male and a 13-year-old girl who developed three and two protruding masses, respectively. The size of each tumor was larger than 5 cm. The histologic appearance showed sharply demarcated tumor lobules composed of eosinophilic shadow cells and basaloid cells. We describe two unusual cases of multiple giant pilomatricomas and review the literature, emphasizing the number and size.
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2/9. Concurrent pilomatrix carcinoma and giant pilomatrixoma.

    A 52-year-old man presented with a large, fungating mass on the inner aspect of his left thigh and a smaller hard mass on the inner aspect of his left knee with normal overlying skin. Both lesions had first been noted by the patient 1 year previously and for the first 6 months had a similar appearance until the thigh mass rapidly increased in size and fungated. pathology of the large thigh lesion showed pilomatrix carcinoma while that of the smaller knee lesion was typical of pilomatrixoma. The pilomatrix carcinoma was widely excised and there has been no evidence of recurrence or metastasis after 3 years. The clinical course of the thigh lesion suggested that pilomatrix carcinoma may arise from a pre-existing pilomatrixoma. On review of the literature, pilomatrix carcinoma of the lower limb may be more likely to metastasize than those on other sites.
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3/9. Guess what! Multiple pilomatricomas and Steiner disease.

    An 18-year-old male adolescent was referred to our department for the presence of multiple skin nodules since the age of 9 years. At the age of 22 months the patient was diagnosed as having myotonic dystrophy (Steinert's disease). So far, nine lesions like these have been excised and histologically analysed, two located on the left arm, one on the neck and one on the scalp. A skin examination revealed three nodular lesions, two localised on the scalp (Fig. 1) and one on the trunk (Fig. 2); the lesions ranged from 0.5 to 3.5 cm, and were hard on palpation and asymptomatic, with elevated, smooth and teleangiectatic borders and a central invagination filled with whitish, firm, granular material. The lesions were all excised. The histological examination of all the biopsy specimens revealed basophilic cells at the periphery, and islets of shadow cells with an unstained central area at the site of lost nucleus (Fig. 3). The shadow cells were surrounded by a granulomatous infiltration composed mainly of giant cells.
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4/9. Proliferating trichilemmal tumor with a malignant spindle cell component.

    BACKGROUND: Proliferating trichilemmal tumors (PTTs) are uncommon exuberant growths of cells derived from the external root sheath. They tend to occur in older women, with a predilection for the scalp. Resection is usually curative. Spindle cell components arising within PTTs are exceedingly rare, with only three known cases previously reported. Of these three cases, one patient died from widespread metastasis. methods: Histological and immunohistochemical examinations were performed on a tumor from a 54-year-old man which, after exhibiting slow growth for 3 years, increased rapidly in size. RESULTS: Microscopic examination revealed cystic lobules of squamous cells without a granular cell layer surrounding amorphous eosinophilic debris within the dermis. Also present was a poorly circumscribed focus of spindle cells with hyperchromasia, pleomorphism, giant cells, and abundant mitoses, including abnormal forms. immunohistochemistry showed the spindle cells to stain positively for vimentin, but negatively for keratin, epithelial membrane antigen and S-100 antibodies. CONCLUSION: A malignant spindle cell component arising within a PTT is a rare neoplasm which has been associated with death from widespread metastasis.
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5/9. A case of perforating pilomatricoma.

    Pilomatricoma is a rare skin neoplasm, most commonly seen in the head and neck region, and occurring in the first two decades of life. It is usually solitary and varies from 0.5 to 2 cm in diameter. Its etiology is unknown. Perforating pilomatricoma is a rare clinical variant that presents as a draining, crusted nodule or ulcer, and is reported to arise faster than the classic pilomatricoma. Herein, we report a case of 35-year-old female, who had a 4-month history of a growing mass on her leg. On physical examination, a 4-cm diameter, asymptomatic, erythematous, ulcerated mass was noted on the left anterio-lateral upper leg. The first histopathological analysis of a punch biopsy from the lesion was reported as basal cell carcinoma. Therefore, the lesion was totally excised. There were shadow cells, squamoid cells, and basaloid aggregations more prominently in the one area in the tumor. In addition, calcification, foreign body giant cells and inflammatory cells were present. Punch or excisional biopsies are preferred as a method of diagnosis for the majority of cutaneous neoplasms. If total excision is not the method of choice, multiple punch biopsies should be made from different areas in large skin tumors for correct diagnosis.
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6/9. Large, rapidly growing pilomatrixoma of the upper eyelid.

    A 41-year-old woman presented with a rapidly growing mass on her upper left eyelid that had first appeared 3 months earlier. The mass was dark red in color, alternating with whitish pseudocyst formations, and measured 3 cm long with a 1-cm base presenting. The rapid growth had caused a mechanical ptosis and bleeding as the result of erosion of the skin covering the tumor. The initial clinical diagnosis suggested a malignant lesion or vascular tumor; excisional biopsy was performed. The eyelid crease was approached for incision and resection as for a blepharoplasty. Pathologic examination yielded a diagnosis of giant pilomatrixoma. Pilomatrixomas are rare in adults and rarely attain such a large size. After 1 year of follow-up, no recurrence of the tumor has been observed.
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7/9. Fine-needle aspiration biopsy of pilomatrixoma: still a diagnostic trap for the unwary.

    Of the 16 cases of fine-needle aspiration biopsy (FNAB) of pilomatrixomas reported since 1982, only 25% were correctly diagnosed prior to excision. The most common pitfall encountered was a false-positive or suspicious diagnosis of a carcinoma. All the reported cases had a polymorphous cellular and noncellular composition on low-power examination, including numerous basaloid cells, ghost cells, squamoid cells, foreign body giant cells, mixed inflammatory cells, keratin clumps, and granular debris which might be easily mistaken for a malignant necrotic background. Amongst these, ghost cells were the critical pathognomonic feature. This paper reported a further finding that had been underemphasized in the literature: masses of refractile orangeophilic keratin clumps representing compact sheets and stacks of ghost cells. Their presence should alert the observer to search for the more diagnostic dispersed forms which otherwise might be inconspicuous and easily overlooked.
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8/9. Malignant melanoma arising in a blue nevus with features of pilar neurocristic hamartoma.

    A 65-year-old man presented with a history of a giant blue plaque of the parietal scalp since childhood. biopsy revealed a cellular blue nevus. The blue nevus was observed for 10 years and thought to be unchanged until a new adjacent lesion was noted. biopsy of the new lesion revealed metastatic malignant melanoma. A wide excision was performed of the original lesion which revealed malignant melanoma arising in a blue nevus. Areas within the blue nevus were consistent with a pilar neurocristic hamartoma, whereas other areas were consistent with a common blue nevus. Subsequent satellite metastases developed, with early metastases resembling blue nevi except for the absence of a stromal component and the presence of hyperchromatic nuclei. Later metastases were typical of metastatic melanoma. This case illustrates the uncommon evolution of malignant melanoma from a blue nevus. The histological features and relationship between melanoma, blue nevus, and pilar neurocristic hamartoma are reviewed.
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9/9. Giant calcifying epithelioma of Malherbe (pilomatrixoma): imaging features.

    We present a case of giant calcifying epithelioma of Malherbe (pilomatrixoma) in the right upper arm of a 62-year-old man. It measured 18 x 12 x 8 cm in size, making it the largest of all the cases reported previously. CT clearly demonstrated a well-defined, subcutaneous mass with amorphous calcifications. The mass showed intermediate signal intensity on T2*-weighted MR images and slight contrast uptake on contrast-enhanced MR images. Histopathologically, this tumor showed no aggressive or malignant nature. The patient is without evidence of recurrence or metastasis 3 years following the resection.
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