Clinical features and outcomes of spitzoid proliferations in children and adolescents.

BACKGROUND: Spitzoid proliferations range from Spitz naevi to melanomas. There are few studies describing clinical features and outcomes in the paediatric population. OBJECTIVES: To determine the clinical features and outcomes of a large paediatric cohort with histopathologically confirmed Spitz tumours. METHODS: This was a retrospective cohort study of patients seen at Boston Children's Hospital who were aged < 20 years and had a histopathological diagnosis of spitzoid proliferation from 1 January 1994 to 23 October 2012. RESULTS: In total 595 patients with 622 spitzoid proliferations were identified (median age 7·4 years, interquartile range 4·6-11·7). Overall 512 proliferations (82·3%) were typical, 107 (17·2.%) were atypical and three (0·5%) were melanomas. The median ages at biopsy were 7·4, 7·2 and 17·2 years, respectively, and there was a significant difference in age at biopsy for patients with typical or atypical proliferations vs. melanoma (P < 0·01). Among samples with positive margins (n = 153), 55% (54 of 98) of typical proliferations, 77% (41 of 53) of atypical proliferations and 100% (two of two) of melanomas were re-excised. Six patients had sentinel lymph node biopsy performed, with three patients demonstrating nodes positive for melanocytic cells. Within a median follow-up of 4·1 years for the full cohort there were no related deaths. CONCLUSIONS: Spitz tumours have strikingly benign outcomes in the paediatric population, although this study is limited by the low number of melanomas and restriction to a single paediatric institution. Aggressive management recommendations should be reconsidered for children and adolescents with banal-appearing Spitz naevi, based on the clinically indolent behaviour in this cohort.

Lymphomatoid papulosis in children.

BACKGROUND: Although lymphomatoid papulosis is well described in adults, the clinical course, prognosis, risk for lymphoma, and recommendations for follow-up have not been established in children. OBJECTIVE: Our aim was to analyze our data on six children with lymphomatoid papulosis and to analyze available information on reported cases from the literature to characterize better lymphomatoid papulosis in childhood and to compare it with adult-onset lymphomatoid papulosis. METHODS: Clinical records, laboratory studies, and histopathologic evaluation of skin biopsy specimens from six children with lymphomatoid papulosis were reviewed. A literature search was also performed and disclosed detailed information on 17 childhood cases. RESULTS: In most cases childhood lymphomatoid papulosis is clinically and histologically similar to lymphomatoid papulosis in adults, but three unusual patterns were identified in our children: first, after initial outbreak, dwindling outbreaks (both in frequency and number of lesions) until the eruption ceased completely; second, lymphomatoid papulosis localized to one area for years before generalizing, and third, presentation of lymphomatoid papulosis with hundreds of lesions. In our children and in those previously reported, response to systemic antibiotics and potent topical steroids was variable, as in adults. All our children to date have remained healthy; the longest period of follow-up is 9 years. However, in previously reported cases two patients with childhood-onset lymphomatoid papulosis had lymphoma as adults. CONCLUSION: Childhood lymphomatoid papulosis may be more likely to resolve spontaneously than adult lymphomatoid papulosis; nevertheless these children may still be at risk for lymphoma and thus need lifelong follow-up.

Association of facial hemangiomas with Dandy-Walker and other posterior fossa malformations.

Cutaneous hemangiomas are common benign tumors of infancy that only rarely are associated with malformations in other tissues or organs. We report nine infants with large facial hemangiomas who also had Dandy-Walker malformations or similar posterior fossa abnormalities. On the basis of the experience with our patients and with those previously reported, we recommend radiographic imaging studies of the brain of infants with large, aggressive facial hemangiomas to rule out posterior fossa defects.

Spirochetes in atrophic skin lesions accompanied by minimal host response in a child with Lyme disease.

Acrodermatitis chronica atrophicans, which has rarely been observed in the United States, is a late skin manifestation of Lyme borreliosis. A 12-year-old girl who spent summers on Cape Cod presented with a 2-year history of hyperpigmentation and atrophy of the skin on the hands, wrists, and ankles. The skin biopsy specimen of an affected area showed mild dermal fibrosis, a few inflammatory cells, and spirochetes morphologically compatible with Borrelia burgdorferi. An IgG antibody response to B. burgdorferi could be elicited by immunoblotting, but not by enzyme-linked immunosorbent assay. We conclude that this patient had chronic Lyme borreliosis manifested only by indolent infection of the skin.

Pigmented basal cell carcinoma and superficial spreading malignant melanoma: an unusual combination.

We report pigmented lesions in 2 white male patients, which were clinically diagnosed as superficial spreading malignant melanoma and histologically showed combined features of superficial spreading malignant melanoma in situ and pigmented basal cell carcinoma. The significance of this association is not known but is most likely very rare.

Histiocytosis-X: in situ characterization of cutaneous infiltrates with monoclonal antibodies.

Cutaneous lesions in three cases of histiocytosis-X were studied by light microscopy, electron microscopy, and immunoperoxidase technics. In each case, Birbeck granule-containing histiocytosis-X cells infiltrated the epidermis and were apposed to lymphocytes. The histiocytosis-X cells and normal Langerhans cells stained with anti-T6 and anti-I1 (Ia-like) antibodies but were negative with anti-T3, anti-T8, anti-M1, and anti-lysozyme antibodies. In addition, the histiocytosis-X cells also stained with anti-T4 antibodies, which react with T-cells associated with helper/inducer phenotype. This study supports the hypothesis that histiocytosis-X cells are abnormal Langerhans cells. The presence of T4/T6-positive cells in cutaneous disease may be a marker for abnormal Langerhans cells.