Sentinel node biopsy in atypical melanocytic neoplasms in childhood: a single institution experience in 24 patients.

INTRODUCTION: Sentinel lymph node biopsy (SLNB) is a controversial but frequently used adjunct to wide excision of difficult-to-diagnose melanocytic proliferations of childhood. We herein report our institutional experience with SLNB in pediatric patients with these lesions, hereafter referred to as 'atypical melanocytic proliferations'. METHODS: Our prospectively collected melanoma database was queried for patients ≤21 years of age status post-SLNB for a diagnosis of atypical melanocytic proliferation in which the diagnosis of melanoma ≥1 mm in depth was considered in the differential diagnosis by one or more expert dermatopathologists and for which no diagnostic consensus could be reached. RESULTS: Of 24 patients identified over 17 years, 7 patients (29%) had a positive sentinel lymph node (SLN). Six SLN-positive patients underwent complete lymph node dissection, with one (14%) having additional nodal involvement identified. With a median follow-up of 4.1 years (range < 0.1 to 14.8 years), all patients showed no evidence of disease. CONCLUSIONS: Despite a significant rate of identification of melanocytes in SLNs of children with atypical melanocytic proliferations, survival appears favorable and controversy surrounding the significance of nodal involvement remains. Further studies with larger numbers of patients and long-term follow-up are needed before the true prognostic value of SLNB in this setting can be determined.

Neutrophilic dermatosis revisited: an initial presentation of lupus?

BACKGROUND: We report 7 patients with a distinct and unusual eruption consisting of a neutrophilic dermatosis in conjunction with lupus erythematosus (LE). The significance of these findings and their relevance to LE are discussed. OBJECTIVE: We aimed to evaluate the significance of eruptive neutrophilic dermatosis in conjunction with LE. METHODS: Seven original cases were collected during 10 years at a tertiary referral center, and were reviewed by a single board-certified dermatopathologist. All patient demographics were tabulated and analyzed. Eleven articles reporting 15 similar cases were obtained from a literature review. RESULTS: Of a total of 7 adult patients, 14% (1 of 7) had a history of LE, whereas 86% (6 of 7) exhibited synchronous initial presentation of neutrophilic dermatosis with LE. Of note, 100% (7 of 7) of the patients exhibited cutaneous lesions on sun-exposed sites. LIMITATIONS: Only 7 of 400,000 cases showed this phenomenon, giving rise to the idea this may be just by chance. CONCLUSIONS: Our data and literature review suggest the existence of a neutrophilic dermatosis distinct from conventional Sweet syndrome that may herald conventional signs and symptoms or represent an initial presentation of cutaneous LE. This neutrophilic dermatosis may share a similar pathogenic mechanism related to ultraviolet exposure.

Osteolipoma of the parapharyngeal space mimicking liposarcoma: a case report.

BACKGROUND: Lipomas are the most common benign neoplasm of the head and neck. However, osteolipomas, a rare variant of lipoma, are uncommon in this location. When they occur, variations in location and radiographic presentation may obscure the diagnosis. METHODS AND RESULTS: A 68-year-old man presented with left jaw pain and numbness in the maxillary (V-1) distribution. A CT angiography of the neck revealed a possible liposarcoma. Embolization of the mass was determined to be unfeasible. Consequently, surgical resection was performed revealing a benign osteolipoma. CONCLUSION: We report a rare case of osteolipoma of the parapharyngeal space. Clinicians should be aware that the clinical and radiological features of patients with head and neck osteolipomas may mimic malignant neoplasms. When given deserved consideration, inappropriate treatment of an otherwise benign lesion may be avoided.

Intravascular schwannoma.

Schwannoma is defined as a benign nerve sheath neoplasm of Schwann cell origin. Cutaneous schwannoma typically manifests along the course of peripheral nerves as a solitary, well-defined, skin-colored nodule within the deep dermis or subcutis of the flexor aspects of the extremities. Schwannoma enlarges slowly and typically follows a benign course, with local recurrence and malignant transformation being exceedingly rare. Although involvement of the vasculature by neurofibroma has been rarely reported, intravascular schwannoma has not been documented to date. We present a unique case of cutaneous schwannoma, as confirmed by histological morphology and immunohistochemistry, within the dermal venous system. Presentation of this case is followed by a discussion of the differential diagnoses of schwannoma, the possible etiologies of the extension of this lesion into the vasculature, and the significance of such a phenomenon.

Primary cutaneous CD30+ T-cell lymphoproliferative disorder presenting as paraphimosis: a case report and review of the literature.

Primary cutaneous CD30+ T-cell lymphoproliferative disorders (PC-CD30+ LPD) as a group are one of the more common types of T-cell lymphoma. More specifically primary cutaneous anaplastic lymphoma (PC-ALCL), one of these lymphoproliferative disorders, is the second most common cutaneous T-cell lymphoma. We report an unusual presentation of PC-ALCL. A 90-year-old, uncircumcised male presented with a 3-week history of painful penile swelling and discharge. The patient was treated with cephalexin and underwent emergent circumcision for paraphimosis. The diagnosis of ALCL was made on microscopic evaluation of the foreskin along with follow-up staging studies. A literature review revealed 31 previously reported cases of penile lymphoma, one of which reported a primary penile CD30+ T-cell lymphoma similar to ours. Only one case report described a lymphoma presenting as paraphimosis. Our case is the second reported case of PC-ALCL of the penis and the first of its kind to present as paraphimosis. Lymphomas must be included in the differential diagnosis of penile lesions and paraphimosis. When present, clinicians should be able to differentiate primary cutaneous lymphoma from lymphomas with secondary skin involvement. All foreskins should be submitted to pathology for proper evaluation of penile lesions.

Basaloid follicular hamartoma.

Basaloid follicular hamartoma is a benign lesion of important consideration because it can be mistaken both clinically and histologically for basal cell carcinoma. The formation of basaloid follicular hamartoma has been linked to a mutation in the patched gene, which is part of the same pathway implicated in nevoid basal cell carcinoma syndrome. While these hamartomas are considered benign lesions, malignant growths have been reported to arise within them, which raises the question, "Is basaloid follicular hamartoma a premalignant lesion?" Correct identification allows for periodic monitoring for malignant transformation, while sparing patients unnecessary surgery. Treatment strategies, including experimental therapies, are reviewed.

Pediatric melanoma: a review.

BACKGROUND: Malignant melanoma is a rare neoplasm in the pediatric population, but its incidence has risen in recent years. METHODS: The literature was reviewed to define the current clinical and pathologic features of pediatric melanoma, highlighting the similarities and differences between adult and pediatric melanoma. RESULTS: Distinctive features of this disease, including frequency and type of genetic abnormalities, predisposing conditions, clinical presentation, stage at diagnosis, prognostic features, and frequency of sentinel lymph node positivity are emphasized. Treatment strategies, extrapolated from adult melanoma trials, are also discussed. CONCLUSIONS: Despite the differences between pediatric and adult melanoma, survival rates are similar and are improving in both populations. Further studies will help delineate the pathogenesis of both adult and pediatric melanoma, with the goal of contributing to early detection and improved survival.