Cases reported "Skin Neoplasms"

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1/79. Secondary reconstruction of a giant congenital lentiginous dermal nevus with serial, large-volume tissue expansion.

    Giant congenital pigmented nevi pose a substantial reconstructive challenge for the treating physician. Due to the increased risk of malignant transformation in such lesions, complete excision with tissue expansion or skin grafting is the generally accepted treatment. These modalities can, however, leave the patient with secondary deformities that also require complex reconstructive procedures. The following case details a patient requiring secondary reconstruction with large-volume tissue expansion 12 years after excision of a giant nevus, and split-thickness skin grafting. This patient illustrates a severe secondary deformity and the usefulness of large-volume serial expansion in such patients.
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2/79. The ABC rule for clinical detection of subungual melanoma.

    BACKGROUND: Subungual melanoma is a relatively rare disease with reported incidence between 0.7% to 3.5% of all melanoma cases in the general population. Unlike the significant improvement in the diagnosis of cutaneous melanoma, the diagnosis of subungual melanoma has shown little, if any, improvement over the years. The widespread adoption of the ABCDs of cutaneous melanoma has helped increase public and physician awareness, and thus helped increase the early detection of cutaneous melanoma; the same criteria cannot be applied to the examination of the nail pigmentation. OBJECTIVE: We reviewed the world literature on subungual melanoma and arranged the available information into a system for the identification of subungual melanoma. This system has to be thorough, easy to remember, and easy to apply by both physician and lay public. A case to illustrate the delayed diagnosis often encountered in the current evaluation of nail melanoma is presented. methods: A thorough review of the world literature on subungual melanoma was undertaken. The important findings of various studies and case reports were compared among themselves and the salient features were summarized. The information was then categorized under the easily recalled letters of the alphabet, ABCD, that have already become associated with melanoma. RESULTS: The most salient features of subungual melanoma can be summarized according to the newly devised criteria that may be categorized under the first letters of the alphabet, namely ABCDEF of subungual melanoma. In this system A stands for a ge (peak incidence being in the 5th to 7th decades of life and african americans, Asians, and native Americans in whom subungual melanoma accounts for up to one third of all melanoma cases. B stands for brown to black b and with breadth of 3 mm or more and variegated borders. C stands for change in the nail band or lack of change in the nail morphology despite, presumably, adequate treatment. D stands for the digit most commonly involved; E stands for extension of the pigment onto the proximal and/or lateral nailfold (ie, Hutchinson's sign); and F stands for family or personal history of dysplastic nevus or melanoma. CONCLUSION: Although each letter of the alphabet of subungual melanoma is important, one must use all the letters together to improve early detection and thus survival of subungual melanoma. Still, as with cutaneous melanoma, the absolute diagnosis of subungual melanoma is made by means of a biopsy.
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3/79. Pediatric skin tumors.

    This article summarizes several malignant childhood neoplasms and benign tumors that can mimic malignancies. Because malignant skin tumors are rare in children, parents and physicians often are not sufficiently suspicious to ensure that an early diagnosis can be made. Many malignant skin tumors have features that suggest a vascular or hemangioma-like lesion. Because hemangiomas occur in 10% of infants, it is often considered prudent to adopt a wait-and-see attitude; however, if the lesion is too firm to be a hemangioma or its growth pattern does not follow that of a typical hemangioma, additional options should be considered. To manage childhood skin malignancies, one needs expert consultation, early biopsy, and correct histopathologic interpretation.
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4/79. Widespread cutaneous vascular papules associated with peripheral blood eosinophilia and prominent inguinal lymphadenopathy.

    A new section entitled "dermatology Grand Rounds at the NIH" appears this month. The idea behind it is to provide a unique look at instructive dermatologic cases being presented at the weekly dermatology Branch Grand Rounds at the National Cancer Institute, National Institutes of health (NIH), Bethesda, maryland. patients seen at the NIH Clinical Center (which includes the NIH inpatient wards and outpatient clinics) are indeed unusual. To be evaluated here, they must be referred by an outside physician and must be placed in a research protocol. In other words, by this criterion, all NIH patients are considered research subjects. The diseases studied at the NIH range from the common to the obscure. Similarly, the dermatologic manifestations of these diseases are equally as diverse. Thus the purpose of this new section is to share some of these fascinating cases with the dermatologic community at large, as well as to provide a glimpse of exciting translational research that has relevance to clinical dermatologists. The format will be simple. Cases will be presented as unknowns, including relevant results from the history, physical examination, and histopathologic analyses. This will be done to challenge the clinicopathologic skills of the readers; authors will make every attempt to make the color clinical and pathologic photographs of the highest quality possible to assist readers in making the correct diagnoses. The discussion will be brief, focusing on the key teaching points of each case. These teaching points will vary from cases to case, covering clinical and histologic information, treatment issues, and/or novel laboratory advances with clinical relevance. Authors will emphasize brevity and clarity in all subsections of the case report, which I hope will promote a good learning experience for dermatology residents and busy practitioners alike. This section will initially be published 4 times annually. Finally, because the section is new to the JAAD, I welcome comments and suggestions to improve the quality of the articles.-Andrew Blauvelt, MD Feature Editor
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5/79. rhinophyma and coexisting occult skin cancers.

    BACKGROUND: Although coexistent tumors have been reported in patients with rhinophyma, few reports have described the coexistence of rhinophyma and an occult infiltrating squamous cell carcinoma (SCC). OBJECTIVE: Preoperatively and during rhinophymaplasty, recognition of subtle changes can suggest an underlying malignancy. methods: A large infiltrating SCC was noted during electrosurgical rhinophymaplasty. Mohs micrographic surgery was performed to clear the tumor. RESULTS: The patient was tumor-free with no evidence of recurrence at 1-year follow-up. CONCLUSION: In the evaluation of changing rhinophyma or subtle changes in tissue noted during rhinophymaplasty, physicians must consider the possibility of an underlying malignancy.
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6/79. Neurocutaneous melanosis: a case of primary intracranial melanoma with metastasis.

    Neurocutaneous melanosis is a rare disorder characterized by the presence of large or multiple congenital melanocytic naevi and benign or malignant pigment cell tumours of the leptomeninges. Distant metastasis is unusual in primary leptomeningeal/intracranial melanomas. We present the case history of an adult male who had multiple primary intracranial melanomas associated with neurocutaneous melanosis (naevus of Ota) in the ophthalmic division of the left trigeminal nerve. Excision of the intracranial tumours was carried out in two stages, but the patient died 2 days after the second operation. autopsy showed multiple metastatic deposits in the liver. Symptoms and signs of raised intracranial pressure, the presence of Ota's naevus, and a dural-based mass or masses should alert the treating physician to suspect a primary leptomeningeal/intracranial melanoma.
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7/79. Pediatric melanoma: confirming the diagnosis with sentinel node biopsy.

    Many pediatric melanoma lesions present at a more advanced stage than those in the adult population. Clinical and histological melanoma mimics, including a subset of Spitz nevi, are difficult to discriminate from melanoma. When dealing with a childhood melanoma, the clinician is likely to be faced with a thick lesion, and one in which the actual diagnosis may even be in doubt. There is a paucity of data to guide the physician in his management of melanoma in this age group, particularly with respect to node status and adjuvant therapy. The authors present two cases of pediatric melanoma in which the novel use of sentinel node biopsy helped confirm the diagnosis of melanoma, determined the need for full lymph node dissection, and guided the use of adjuvant interferon therapy.
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8/79. Zosteriform Darier's disease versus acantholytic dyskeratotic epidermal nevus.

    patients with keratotic lesions distributed in a unilateral, linear, zosteriform or localized pattern and revealing histologic features of dyskeratotic acantholysis have been reported. There is still some controversy regarding the appropriate nosologic placement of this entity. Some believe it represents a localized form of Darier s disease, while others argue it is a variant of epidermal nevus. We report a case of a 42-year-old physician who presented with a 15-year history of an asymptomatic eruption that had been diagnosed as "chronic zoster." Physical exam revealed hyperkeratotic papules and plaques in a dermatomal distribution. The controversy regarding the correct nosologic placement of such a patient is discussed.
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9/79. Cowden disease.

    Cowden disease represents an unusual, but unique syndrome which can be recognized most consistently by the development of characteristic verrucous, keratotic, papular, and nodular lesions about facial orifices, on the oral mucosa, and over the dorsal surfaces of the forearms and hands. The cause of these diverse hyperplastic changes is not known. Recognition of these lesions as signs of more extensive disease should alert the physician to examine the thyroid gland, breasts, female reproductive tract, GI tract, and skeleton for evidence of the associated changes we have enumerated. Because of the tendency for lesions of the thyroid, breast, and intestines to undergo malignant change, these patients require close observation and evaluation.
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10/79. Adjuvant therapy of melanoma.

    In 2001, the American Joint Committee on Cancer melanoma Staging Committee proposed and created a new staging system for melanoma. This new system will become official in 2002, with the publication of the sixth edition of the AJCC Cancer Staging Manual. The new system identifies significant prognostic variables in patients with melanoma and validates them in an analysis of 17,600 patients, making it possible to precisely determine the patient's chance for survival In light of physicians' ability to determine with more precision which patients are at high risk for melanoma recurrence, they face the dilemma of which, if any, surgical adjuvant therapy to choose. Alpha-interferon is the only agent approved for adjuvant therapy of melanoma in the united states, but its questionable benefits and substantial side effects make it hard to justify recommending it to patients. Discussion of trials of high- and low-dose interferon is presented here. The author's group has conducted trials of granulocyte-macrophage colony-stimulating factor (GM-CSF [Leukine]) as surgical adjuvant treatment of patients at high-risk for melanoma recurrence. One of the most important activities of GM-CSF is its ability to activate macrophages and cause them to become cytotoxic for human melanoma cells, at doses low enough to avoid the toxicity associated with other cytokines. The author presents promising trial results, discusses GM-CSF in other malignancies, and includes discussion of tumor vaccines, biochemotherapy, and other agents being studied as adjuvant therapy of melanoma. It is hoped that these newer approaches will result in therapies that are more effective and less toxic than interferon.
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