RESUMO
Spiradenocarcinoma (SC) is a very rare malignant skin adnexal tumor with sweat gland differentiation that develops from a pre-existing spiradenoma, cylindroma, or hybrid tumor called spiradenocylindroma, or arises de novo. We present two exceptionally rare SC cases showing sarcomatous differentiation; we also discuss the clinicopathologic features of SC, as well as its differential diagnoses and available therapeutic modalities. Given the aggressive behavior of SC, rapid diagnosis and complete removal of the tumor with tumor-free margins is mandatory. Owing to the marked morphological heterogeneity of individual SC cases, dermatopathologists must be familiar with the different possible histopathologic manifestations of this neoplasm.
Assuntos
Adenocarcinoma/diagnóstico , Metaplasia/patologia , Sarcoma/patologia , Neoplasias Cutâneas/patologia , Neoplasias das Glândulas Sudoríparas/patologia , Acrospiroma/complicações , Acrospiroma/patologia , Adenocarcinoma/patologia , Adenocarcinoma/cirurgia , Adulto , Idoso , Carcinoma Adenoide Cístico/complicações , Carcinoma Adenoide Cístico/patologia , Diferenciação Celular , Diagnóstico Diferencial , Feminino , Humanos , Imuno-Histoquímica/métodos , Perda de Seguimento , Margens de Excisão , Sarcoma/diagnóstico , Neoplasias Cutâneas/cirurgia , Coloração e Rotulagem/métodos , Neoplasias das Glândulas Sudoríparas/cirurgiaRESUMO
Background. Surgical treatment of malignant pelvic bone tumors can be very challenging. The objective of this retrospective study was to evaluate the oncological as well as the clinical and functional outcome after limb salvage surgery and biological reconstruction. Methods. The files of 27 patients with malignant pelvic bone tumors, who underwent surgical resection at our department between 2000 and 2011, were retrospectively analyzed (9 Ewing's sarcoma, 8 chondrosarcoma, 4 osteosarcoma, 1 synovial sarcoma, 1 malignant fibrous histiocytoma, and 4 carcinoma metastases). Results. After internal hemipelvectomy reconstruction was performed by hip transposition (n = 16), using autologous nonvascularised fibular graft (n = 5) or autologous iliac crest bone graft (n = 2). In one patient a proximal femor prothetis and in three patients a total hip prosthesis was implanted at the time of resection. The median follow-up was 33 months. Two- and five-year disease-specific survival rates of all patients were 86.1% and 57.7%, respectively. The mean functional MSTS score was 16.5 (~55%) for all patients. Conclusion. On the basis of the oncological as well as the clinical and functional outcome, biological reconstruction after internal hemipelvectomy seems to be a reliable technique for treating patients with a malignant pelvic bone tumor.
RESUMO
This paper deals with bilateral vascularized fibular grafts (BVFG) as a method for reconstruction of metadiaphyseal defects of the femur and tibia in young patients suffering from malignant bone tumors of the lower limb. This reconstructional technique was used in 11 patients undergoing metadiaphyseal resection of lower limb malignant bone tumors. All patients with Ewing's sarcoma and osteosarcoma had multimodal treatment according to the EURO-E.W.I.N.G 99 or COSS-96 protocol. Median FU was 63 months. None of the patients experienced local recurrence during FU. 2 patients died due to distant disease during FU. Full weight- bearing was permitted after a mean of 8 months. The median MSTS score was 87%. Complications occurred in five patients. None of the complications led to failure of the biological reconstruction or to amputation. Biological reconstruction of osseous defects is always desirable when possible and aims at a permanent solution. Good functional and durable results can be obtained by using BVFG for the reconstruction of metadiaphyseal defects of the femur and tibia. Radiotherapy in the multimodal setting increases the risk for graft or fixation failure.