Infantile digital fibromatosis: a rare tumour of infancy. Report of five cases.

Infantile digital fibromatosis (IDF) is a rare tumour of infancy with a typical clinical presentation and characteristic histopathological findings. Despite an alarming appearance, IDF does not cause deep infiltration or metastasis. The traditional approach of surgical resection was recently challenged by increasing evidence of self regression in months or years. We describe the clinical history of five patients with IDF that were followed in our departments for 5 years.

Long-term follow-up of metil aminolevulinate (MAL)-PDT in difficult-to-treat cutaneous Bowen's disease.

Bowen's disease (BD) is a form of intraepidermal squamous cell carcinoma, which is clinically characterized by gradually enlarging, well-demarcated erythematous plaques with irregular borders and surface crusting or scaling, affecting primarily the elderly. BD often presents with lesions difficult to treat with standard therapy as surgery, cryosurgery, or 5-fluorouracil (5-FU) for the risk of significantly poor cosmetic outcome, failure rate, and adverse events, related mainly to the age of the patients. Topical PDT with methyl aminolevulinate (MAL) represents a valid and approved therapy for BD lesions in many cases, especially for lesions located at poor healing sites or for large patches of disease, due to its high efficacy coupled with good tolerability and tissue-sparing attitude. In this study, we sought to investigate the efficacy, safety, and cosmetic outcome of MAL-PDT in a series of patients with BD lesions which were challenging to treat for clinical, surgical, and patient-related reasons, such as size of the lesion, difficult surgical approach for anatomical sites, or age of patients and request of the best cosmetic result. We also performed a long-term follow-up to assess recurrence rates and eventual late-onset adverse events.

Cutaneous T-cell/histiocyte-rich B-cell lymphoma: a case report and review of the literature.

BACKGROUND: T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) primarily presenting on the skin is an extremely rare entity with only sporadic cases reported in the literature. METHODS: We here report an extraordinary case of primary cutaneous THRLBCL with self-healing and 24 months of follow-up. RESULTS: The lesion was a dermohypodermal/subcutaneous circumscribed ulcerated nodosity. Histological examination with immunohistochemical, molecular analysis and comparative genomic hybridization were performed. A complete staging was negative for secondary involvement. CONCLUSION: Our case is remarkable because it is the second well-documented primary cutaneous THRLBCL in which we observed a complete self-regression of skin lesions without recurrences or dissemination of the disease. According to the literature, we highlight that the tumoral microenvironment, in our case, could play a relevant role in stopping lymphoma growth. Furthermore, this case supports the observation that THRLBCL primarily presenting on the skin shows an overall good prognosis.

Cutaneous extranodal NK/T-cell lymphoma: a clinicopathologic study of 5 patients with array-based comparative genomic hybridization.

Extranodal natural killer/T-cell (ENK/T) lymphoma is a rare neoplasm, subcategorized into ENK/T-nasal (ENK/T-N) and ENK/T-nasal type (ENK/T-NT) lymphomas. ENK/T-NT lymphoma with initial presentation in the skin is known as primary cutaneous ENK/T-NT (PC-ENK/T-NT) lymphoma. The aim of this study was to investigate pathogenesis, genomic alterations, and prognosis of cutaneous ENK/T lymphomas to provide further insights into clinicopathologic features and genetic mechanism of lymphomagenesis. A retrospective case study of 5 white patients affected by ENK/T lymphoma (4 PC-ENK/T-NT and 1 ENK/T-N with cutaneous involvement) was performed. Most of the cases presented with multiple nodules and ulcerations localized on the extremities. A considerable percentage had disease in advanced stage with a 12-month survival rate of 40%. Genomic alterations were detected by array-based comparative genomic hybridization that showed gains of 1q, 7q and loss of 17p in the cases of PC-ENK/T-NT lymphomas and gain of 7q and loss of 9p, 12p, 12q in the case of ENK/T-N lymphoma. In conclusion, ENK/T lymphoma is a very aggressive entity, and, in our cases, the exclusively cutaneous presentation was not associated with a better prognosis. The results of our array comparative genomic hybridization analysis could be useful to better define the different ENK/T lymphoma subgroups with cutaneous involvement.

Familial syringoma: report of two cases with a published work review and the unique association with steatocystoma multiplex.

Syringoma is a benign neoplasm of eccrine origin. Clinically, it manifests as small skin-colored to yellowish soft papules usually localized around the eyes and on the upper cheeks of middle-aged women. Familial cases have rarely been reported and may be inherited as an autosomal dominant trait or result from either germ line or somatic mutations. Syringoma can coexist with various conditions, notably Down syndrome. Herein, we report a family with multiple syringomas affecting members of three following generations and describe in detail a 36-year-old woman and her 17-year-old son. In the latter, steatocystoma multiplex, which is regarded as a benign cystic neoplasm of the folliculosebaceous unit or a nevoid malformation differentiated in the direction of the sebaceous duct, was associated. Acral distribution of steatocystoma multiplex and its presentation as subcutaneous nodules in this patient were unique.