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• W2023372463 abstract "Patients undergoing bone marrow transplantation for haematopoietic malignancies have an increased risk of developing secondary malignancies. It has been estimated that the incidence of secondary cancer is 4.2 times higher than of primary cancer in the general population. Potential risk factors associated with the development of secondary cancers in bone marrow recipients include the use of an alkylating agent in therapy, previous total-body irradiation, severe acute graft-versus-host disease (GVHD) from grade II to grade IV, treatment of acute GVHD with either antithymocyte globulin or CD3 receptor antibody, and possibly chronic GVHD [1].Secondary solid tumours tend to develop at 11–62 months after transplantation and include mucocutaneous tumours (squamous cell carcinoma of tongue, oral cavity or skin; malignant melanoma or basal cell carcinoma), central nervous system tumours, gastro-intestinal, genital or mammary and undifferentiated carcinomas or sarcomas.We report a 50-year-old woman who developed a Merkel cell carcinoma (MCC) with secondary lymph node involvement 7 years after autologous bone marrow transplantation for non-Hodgkin’s lymphoma.A 50-year-old woman was referred to our Department in September 1998 for evaluation of a rapidly growing mass on the left cheek that had appeared 5 months before. Five months prior to our examination a papular lesion on the same location was excised and the diagnosis of neuro-endocrine carcinoma of the skin (MCC) was established.The medical history revealed essential hypertension, depression since the age of 43 years and a centrocytic/centroblastic non-Hodgkin’s lymphoma stage IIIA diagnosed in 1988, treated with chemotherapy (6 cycles CHOP) in 1988. In 1992, after a lymph node recurrence, an autologous bone marrow transplantation was performed. In 1993, a lymph node mediastinal recurrence was detected and she was treated with radiotherapy. After a 6-year follow-up period, the patient remained in complete remission without treatment.Physical examination revealed a symptomless woman with a firm, non-adhering, shiny and flattened surface nodule, 6.5 × 6 cm in diameter, on the left cheek (fig. 1). The lesion adhered to the underlying skin and deeply infiltrated the cheek. No regional lymph nodes were detected and the rest of the physical examination was unremarkable.Several cutaneous biopsies were performed. A nodular growth involving the entire dermis and extending to the subcutaneous tissue and to the underlying striated muscle was observed. The nodule was composed of small pleomorphic basophilic cells (fig. 2) that were positive for cytokeratin 20, neuron-specific enolase and negative for leucocyte common antigen. Laboratory investigations (haematological and biochemical blood parameters) were normal.The patient underwent radical excision, left cervical functional lymphadenectomy and surgical reconstruction. Additional treatment with local radiotherapy (60 Gy total dose) on the left cheek area was administered.In February 1999, the patient noticed a left axillary mass and an infraclavicular lymphadenopathy. An excisional biopsy demonstrated local and lymph node recurrence of MCC. Thoracic, abdominal and cervical CAT scan did not reveal further involvement.Four courses of chemotherapy with cyclophosphamide and Adriamycin and local radiation therapy at a total dose of 38 Gy were administered. Nevertheless, a local progression of the disease was observed.MCC or neuro-endocrine carcinoma of the skin is an aggressive carcinoma with high rates of local recurrence and distant metastasis (36%) [2]. Clinically, it manifests itself as a solitary dome-shaped, red, violaceous nodule or indurated plaque with a shiny surface often with underlying telangiectasies. The most common location for MCC is sun-exposed skin of elderly patients, particularly in the head and neck area (53%) and on the extremities (35%). It has been suggested that actinic damage and ultraviolet light may contribute to the development of MCC. Concomitant development of squamous cell carcinoma, basal cell carcinoma and sweat gland carcinomas has repeatedly been observed in patients with MCC.Several cases of MCC arising in immunosuppressed patients have been reported [3]. MCC has been reported to develop in renal or cardiac transplant recipients [4, 5, 6]treated with corticosteroids and/or cyclosporin [4]and/or azathioprine [7], in patients with chronic lymphocytic leukaemia [8], rheumatoid arthritis or systemic lupus erythematosus [9]under chronic immunosuppressive treatment and rarely in HIV-positive patients [10].MCC arising in an immunosuppressive setting seems to affect a younger group of patients. No evidence of solar damage is usually observed and the lesions tend to develop on the trunk and extremities. The time from initiation to immunosuppression to the development of MCC ranged from 4 to 20 years. Rarely, spontaneous regression of the tumour after withdrawal of immunosuppression has been reported [11].Some authors have suggested that immunocompromised patients with overt haematological malignancies such as leukaemia appear more susceptible to MCC and develop more rapidly progressive lesions at a younger age. It has been estimated that chronic lymphatic leukaemia seems to be 1,000 times more frequent in patients with MCC than in the general population. MCC has also been described in patients previously treated for Hodgkin’s disease or non-Hodgkin’s lymphoma. As far as we know, only one previous case report of MCC developing after bone marrow transplantation has been published [12].Adult recipients of bone marrow transplants are at a significantly higher risk for developing cancers than are members of the general population [13]. In our patient, only the previous irradiation could be incriminated as a possible cofactor implicated in the development of MCC. Our observation reinforces the role of immunosuppression in the development and pathogenesis of MCC and illustrates the possible association of MCC with bone marrow transplant recipients." @default.
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• W2023372463 title "Merkel Cell Carcinoma Developing after Bone Marrow Transplantation" @default.
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