DERMATOPATHOLOGY CASES: Self-Assessment Cases: Editor - Dr Sampurna Roy MD

Digital Images of interesting cases that will include the full spectrum of Dermatopathology, presented in the form of quiz.

The answer of the cases include related links and recent abstracts of articles.








Monday, December 14, 2009

Answer of Dermatopathology Case 15


Cylindroma

Visit: Pathology of Cylindroma
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Abstract:

Cylindroma of the breast: a case report and review of the literature. Diagn Pathol. 2009 Sep 2;4:30.
ABSTRACT: Cylindroma of the breast is a very rare lesion which is morphologicaland immunophenotypically identical to benign dermal cylindroma. We report a breast cylindroma in a previously healthy 62 year old female detected through anational breast screening program. The patient had no significant family or past medical history, and specifically no history of breast or skin diseases. The tumor consisted of well circumscribed islands of epithelial cells surrounded by adense membrane material, and focally containing hyaline globules. At low power the islands of tumour cells formed a "jig-saw" pattern, which is typical of cylindroma, but was present within normal breast parenchyma and no had directconnection with the overlying skin. Two distinct cell populations, smaller peripheral basaloid cells and larger central cells with vesicular chromatin, were highlighted by immunohistochemistry for p63 and cytokeratin-7 respectively. Immunohistochemistry for ER, PR, and Her2/neu was negative in tumour cells. We discuss the nine previously reported cases and the distinction of breast cylindroma from adenoid cystic carcinoma, the main differential diagnosis.

Management of whole-scalp tumour: the Poncet-Spiegler cylindroma or turban tumour.Rev Stomatol Chir Maxillofac. 2009 Apr;110(2):109-12. Epub 2009 Mar 27.
INTRODUCTION: The Poncet-Spiegler cylindroma (PSC) is a benign annexal cutaneous tumor which preferentially develops on the scalp, neck, or forehead. Localizations may be isolated or multiple and often affect the young adult. The lesions grow progressively. When the scalp is completely involved, it presents as a "turban tumor". Treatment is surgery and may be difficult when the tumor isextended. We report the management of turban tumor. OBSERVATION: A 25 year-old female patient was first treated by partial scalp nodule exeresis and histology documented a PSC. She was lost to follow-up. But 9 years later, she was managedfor a turban like PSC. The treatment was a complete scalp exeresis and secondary reconstruction with a skin graft. Follow-up was uneventful with a progressive functional and cosmetic improvement and after 2 years, there was no relapse. DISCUSSION: As for most tumors, management depends on the size of the PSC. An aggressive surgical treatment must be considered if the PSC is extended. In case of turban tumor, total scalp exeresis and secondary reconstruction with a skin graft is recommended.

Benign cylindroma causing transcalvarial invasion in a patient with familial cylindromatosis. Clin Neuropathol. 2007 May-Jun;26(3):125-30.
OBJECTIVE: To describe a case of scalp cylindroma without features of malignancy invading through the skull and dura, and producing massive intracranial extension. Tumors of epidermis and epidermal appendages rarely show bony invasion, but invasive tendency in some tumor types has been associated with increased TP53 expression. PATIENT AND METHODS: Patient with familial cyindromatosis (Brooke-Spiegler syndrome) who had undergone numerous previous surgical excisions over the past 30 years of his scalp cylindromas. Light microscopic and immunohistochemical characterization of resected tumor, with TP53 immunostaining in the invasive tumor was compared with that seen in five other cutaneous, non-invasive cylindromas. RESULTS: Tumor showed no increase in mitotic rate or increased immunostaining for TP53. CONCLUSION: Multiple previous surgeries down to pericranium may have contributed to local weakening of tissues and facilitated transcalvarial invasion. While an uncommon occurrence, both benign and malignant cylindromas have the capacity to invade bone, particularlyin patients with the familial syndrome.