Salve G. Ronan

Atypical Spitz nevi/tumors : Lack of consensus for diagnosis, discrimination from melanoma, and prediction of outcome

The biological nature of Spitz nevi/tumors and their diagnostic distinction from, or relationship to, melanoma remain unresolved issues. In this report, a series of 30 melanocytic lesions removed from 28 patients, including atypical Spitz nevi/tumors and metastasizing Spitzoid tumors/melanomas, were evaluated by a panel of dermatopathologists to evaluate interobserver diagnostic concordance and to assess the prognostic power of histological criteria. For inclusion in the study, each lesion had to display some criteria for the Spitz nevus, and in addition one of the following was required: (1) definitive clinical outcome such as metastasis or death of disease, or (2) long-term follow-up if the patient remained disease free. Each lesion was reviewed independently and blinded as to the clinical data by 10 pathologists, who categorized them as (1) typical Spitz nevus/tumor, (2) atypical Spitz nevus/tumor, (3) melanoma, (4) tumor with unknown biological potential, or (5) other melanocytic lesion. There was limited discussion of criteria before the review. Evaluation of 17 Spitzoid lesions yielded no clear consensus as to diagnosis; in only one case did six or more pathologists agree on a single category, regardless of clinical outcome. Notably, however, some lesions that proved fatal were categorized by most observers as either Spitz nevi or atypical Spitz tumors. Conversely, seven or more pathologists scored 13 lesions as melanoma. These results illustrate (1) substantial diagnostic difficulties posed by many Spitz tumors, especially those with atypical features, even among experts, and (2) the lack of objective criteria for their distinction from melanoma and for gauging their malignant potential. Nevertheless, our observations do suggest that a biological relationship exists between the Spitz nevus/tumor and melanoma.

Panniculitis associated with cutaneous T‐cell lymphoma and cytophagocytic histiocytosis

A 36‐year‐old woman had a 6‐year history of recurrent panniculitis with development of an angiocentric and angiodestructive cutaneous T‐cell lymphoma (CTCL) of the helper cell phenotype. She subsequently developed a rapidly progressive fatal syndrome characterized by cytophagocytic histiocytosis and hyperlipidaemia. Cytophagocytic histiocytosis has previously been reported in association with panniculitis, malignancy and infection, but not with CTCL and the precise relationship between panniculitis, CTCL, cytophagocytic histiocytosis and hyperlipidemia is unclear.

Histologic criteria for the diagnosis of mycosis fungoides : proposal for a grading system to standardize pathology reporting

Background: The histological diagnosis of early lesions of mycosis fungoides (MF) is often difficult for dermatopathologists and prior studies have shown a low agreement rate among pathologists. An important reason for such difficulty may be the lack of specific histological criteria.

Melanoma in childhood and adolescence: Clinical and pathological features of 48 cases

Forty-eight cases of melanoma occurring in patients under 20 years of age were reviewed from a 23-year period at a single center. Fourteen of the patients were preadolescent children and 44 were Caucasian. Histological review of 44 available primary tumors showed only superficial spreading and nodular types. Thickness ranged from 0.23 mm to 8.50 mm, with a median of 1.03 mm. Ulceration was present in 7%, necrosis in 35%, evidence of regression in 16%, and antecedent nevus in 49% of the cases. The overall 5-year survival is 77%, with a median follow-up of 48 months. There is no detectable survival difference between preadolescent children and adolescents. Several treatment failures occurred after improper biopsy and/or inaccurate original diagnosis of Spitzs nevus. Of 38 stage I and II patients given definitive surgical treatment by the authors, the 5-year survival is 90%. Although histological confusion with Spitzs nevi occasionally occurs, melanoma in this age group can be treated with good results.

Merkel cell carcinoma: In vitro and in vivo characteristics of a new cell line*

BACKGROUND Few studies exist that describe Merkel cell carcinoma (MCC) growth characteristics in vitro, in vivo, or both. OBJECTIVE Our purpose was to evaluate the pathologic features of MCC implanted into athymic mice and to determine cytogenetic abnormalities in the established cell line. METHODS Tumor tissues from a patient with MCC were grown in culture. Cultured cells were karyotyped and inoculated subcutaneously into athymic mice. Nude mouse tumors were re-implanted into other athymic mice. Tissues from the primary skin tumor and the nude mouse tumor were processed for light and electron microscopy and immunocytochemistry. RESULTS The cell line showed a doubling time of 64.8 hours. Xenografts of 4 x 10(6) cells produced tumors in athymic mice with a doubling time of 16.1 days. The nude mouse tumors showed pathologic features similar to those of the primary skin tumor. Cytogenetic studies showed a t(1;17) (p36;q21) translocation in 100% of the cells. CONCLUSION MCC implanted into athymic mice retained the pathologic features of the primary skin tumor and behaved aggressively. The t(1;17) (p36;q21) translocation may be a marker of an aggressive phenotype.

Primary cutaneous phaeohyphomycosis: report of seven cases

We report seven cases of primary cutaneous phaeohyphomycosis. There were five males and two females, ranging in age from 42–65 years (mean 57.7 years). Two patients were otherwise healthy, but five were immunocompromised. One patient had rheumatoid arthritis and was on oral prednisone; two were renal transplant recipients, one was a heart transplant recipient, and the fifth had dermatomyositis. No history of trauma was elicited from any of the patients, but in two cases, foreign material was seen in the tissue sections. All lesions were oil the extremities. In two cases, tissues were cultured, and these grew Exophiala jean‐selmei. The others were not cultured because fungal infection was not clinically suspected. No systemic disease developed in any of the cases, and all were cured by the simple, complete excision of the lesions.

Molecular analysis of 1p36 breakpoints in two Merkel cell carcinomas

Merkel cell carcinoma (MCC) is a rare aggressive neuroendocrine tumor of the skin. Only little information is available on the genetic alterations occurring in this tumor. Cytogenetic studies thus far have not shown recurrent chromosomal changes, although various structural chromosome 1 rearrangements, including deletions, often leading to loss of distal 1p material appear to be frequent. We report on fluorescence in situ hybridization and loss of heterozygosity analyses of an MCC tumor and MCC cell line UISO. The present study has shown that two distinct regions in the most distal band 1p36 on the short arm of chromosome 1 can be implicated in MCC. One region at 1p36.3 was delineated by a distal deletion in the MCC tumor as a result of an unbalanced translocation, resulting in loss of all markers distal to ENO1. This region was previously shown to be deleted in different tumor types including neuroblastoma. In cell line UISO an insertion in 1p36.2 was identified. The insertion breakpoint indicates a second, more proximal, region on 1p involved in MCC. The insertion breakpoint was mapped within a cluster of repetitive tRNA and snRNA genes and thus could coincide with the constitutional 1p36 breakpoint previously reported in a patient with neuroblastoma. Genes Chromosomes Cancer 23:67–71, 1998.

Dissecting cellulitis of the scalp

Dissecting cellulitis of the scalp or perifolliculitis capitis abscedens et suffodiens is a rare, chronic, progressive, suppurative disease of the scalp of unknown etiology. It is characterized by painful nodules, purulent drainage, burrowing interconnecting abscesses, and cicatricial alopecia. The pathogenesis is unknown, although it is probably related to follicular occlusion, secondary infection, and deep inflammation. Black men in their second to fourth decade are predominantly affected. Treatment varies from systemic antibiotics to incision and drainage, x-ray epilation of the affected areas, systemic steroid administration, and surgical excision. Our experience with four patients with extensive scalp disease is presented. Wide excision of the affected areas and split-thickness skin graft are favored as our treatment of choice.

Disseminated strongyloidiasis presenting as purpura

We report a patient with disseminated strongyloidiasis who was being treated with steroids for cerebral edema caused by brain metastases from urinary bladder carcinoma. He had extensive purpura involving the abdomen, arms, and thighs. A skin biopsy specimen showed numerous larvae of Strongyloides stercoralis. Subsequently, rhabdoid larvae of S. stercoralis were isolated in the stool and the sputum. The patient died 2 days later despite thiabendazole therapy.

Plasma cell myeloma in a patient with a cutaneous T‐cell lymphoma

A case is reported, believed to be the first, of a patient with a long‐standing cutaneous T‐cell lymphoma, in whom disseminated plasma cell myeloma subsequently developed shortly before the patient died. It is postulated that, rather than to a chance association, the second B‐cell neoplasm evolved under the sustained inducer‐cell activity of the malignant lymphocytes of the cutaneous T‐cell lymphoma.