Management of ocular adnexal and motility problems associated with midfacial fractures.
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It has been debated whether malignant transformation of trichoblastoma occurs. The concept was recently forwarded that basal cell carcinoma is as a malignant neoplasm of follicular germinative cells and should be named trichoblastic carcinoma to show its relationship to trichoblastoma. Almost all basal cell carcinomas are low-grade malignant neoplasms and develop metastases only very rarely, and if so, only after very long duration and untreated growth. Only rare basal cell carcinomas arise in trichoblastomas. Up to now there have only been two reports of high-grade trichoblastic carcinoma arising in trichoblastoma, showing systemic metastatic spread and death. We add two further cases of trichoblastic carcinoma with anaplastic nuclei, arising in trichoblastoma. One of the tumors arose in a small nodular trichoblastoma on the right forearm of an 84-year-old male patient. The other one was a trichoblastic carcinoma at the base of a trichoepithelioma on the right thigh of an 87-year-old woman with Brooke-Spiegler syndrome. Our cases emphasize that high-grade trichoblastic carcinoma develops via malignant transformation of trichoblastoma, and is very rare.
PURPOSE: To report the clinical features in a series of patients with perineural spread of squamous cell carcinoma involving periorbital nerves that presented with clinical and/or imaging evidence of pseudo-cystic transformation along the involved nerves. METHODS: A noncomparative, retrospective chart review of the clinical and imaging findings of patients attending a regional orbital surgery department between 1998 and 2005, presenting with a pseudo-cystic orbital mass on clinical examination and/or imaging, which proved to be due to perineural squamous cell carcinoma on histopathology. RESULTS: The study included 8 male patients with a mean age at referral of 66 +/- 11 years. All cases had associated cutaneous squamous cell carcinoma involving the face or scalp, and, in 4 cases, the primary tumor could be identified in the vicinity of the affected orbit. The duration of the symptoms varied from 5 weeks to 9 years (24 +/- 36 months). Altered sensation, including formication and hypo-esthesia in the V1 and V2 trigeminal division, motor nerve palsies, and ptosis in conjunction with a palpable periorbital mass, were the most common presentations. The cystic tumor deposits were assessed histologically with both hematoxylin and eosin and immunoperoxidase stains (S100 protein for neural structures identification and MNF116 as a keratin marker). This demonstrated malignant squamous epithelium both within and around the wall of the tumor deposit and, in continuity, within the nerve running through the lesion. Some nerves showed substantial areas of fibrosis, representing obliteration of the nerve structure caused by involvement by tumor. Treatment modalities included surgical debulking, exenteration, radiotherapy, and combined chemo-radiotherapy. Mean follow-up was 29 +/- 23 months. Eight patients (87.5%) remain alive, and five of them show no evidence of disease. One patient died after progression of the malignancy. CONCLUSIONS: The presence of a cystic lesion in association with sensory or motor deficit in the periorbital region should suggest a diagnosis of perineural spread from a cutaneous squamous cell carcinoma. Because these patients may present to the ophthalmologist first, an awareness of this entity is of critical importance to avoid the delayed diagnosis seen in half of our cases.
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Nearly 100 mouse mutations have been described as causing some type of abnormality of the skin or hair. As only a few of these mutations have been studied in detail, they remain an untapped resource for furthering knowledge of basic cutaneous physiology and understanding the pathophysiology of analogous diseases in humans. Several diverse murine mutations are discussed. These include "asebia," a mildly hyperkeratotic disorder with sebaceous gland hypoplasia; "ichthyosis," an example of abnormal hair growth associated with hyperkeratosis; "rhino" and "hairless," two related examples of congenital follicular malformations; and "flaky skin", a potential animal model of eruptive psoriasis.
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A patient with a sclerosing-sweat-duct carcinoma of the upper lip is reported. Histologically the tumour showed solid islands and strands of squamous cells and sparse small ductal structures, some of them containing central cores of dense eosinophilic keratin. All this was embedded in a sclerotic stroma. These features, in addition to positive immunoreactivity for carcinoembryonic antigen (CEA) in the lumina of small ducts, and the presence of S-100 protein-positive cells in some cords and ducts, are consistent with the notion that this tumour exhibits differentiation toward eccrine sweat structures.
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Cyclosporin is currently considered a new and interesting drug in veterinary dermatology for the treatment of immune-mediated skin diseases, and a safe and effective alternative to immunosuppressive therapy with glucocorticoids. The authors report a case of granulomatous folliculitis and furunculosis and of sebaceous adenitis in two cats and a case of alopecia areata in a dog, successfully controlled with cyclosporin.
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