Abstract
Background: Head and neck sarcomas are very rare tumors, accounting less than 1% from all malignancies of this region. They represent a heterogeneous group of tumors with distinct prognostic features. There have been significant improvements in characterizing these sarcomas using traditional morphologic assessments and immunohistochemical analysis, but treatment management is still a big challenge. Prognostic factors are essentially tumor grade, margin status and tumor size. Surgery remains the mainstay of treatment, despite the anatomic constraints, followed by radiation therapy. Aim: to show the rarity of the head and neck localization, and describe epidemiologic, clinico-radiological, histological features, treatment management and the evolution of these tumors. Methods: It is a retrospective descriptive study conducted in the department of medical oncology at Hassan II University Hospital between January 2007 and December 2011, including all patients with histologically proven melanoma of the anorectal area. Results: 10 cases were collected, that represented 7 % from all sarcomas reported at the study period. Five men and 5 women, the mean age was 34 years (range: 17-65years). Local imaging was performed by MRI in six cases and CT scan in 4 cases. It had showed locally advanced disease in 7 cases and among them 6 patients had a metastatic disease at the staging. Lung was the site of predilection of metastases. Histological analysis with the support of immunohisto-chemestry showed diverse histological subtypes. For the 3 cases with localized stages, the mainstay treatment was based on surgery followed by radiation therapy in one case with unclear margins. For metastatic stages, first line of chemotherapy was mainly based on: doxorubicin, ifosfamide, and cisplatine for osteosarcoma. Evolution was marked by disease controlee and remission in 2 patients who underwent surgery with clear margins. Unfortunately by recurrence in patient operated with unclear margins and by rapid progression for metastatic stages leading to death. Conclusion: Adult head and neck sarcomas are very rare tumors with poor prognosis, high aggressiveness and rate of recurrence. They are frequently diagnosed at advanced stages. Treatment management is still a big challenge given to the difficulty of surgery and modest response to chemotherapy.
Highlights
Head and neck sarcomas are rare tumors, accounting for only 1% of all head and neck malignancies and 5% of all sarcomas [1,2]
By recurrence in patient operated with unclear margins and by rapid progression for metastatic stages leading to death
Treatment management is still a big challenge given to the difficulty of surgery and modest response to chemotherapy
Summary
Head and neck sarcomas are rare tumors, accounting for only 1% of all head and neck malignancies and 5% of all sarcomas [1,2]. The treatment depended on histological type, grade, stage and operative considerations according to the site. They are characterized by high rate of local recurrence and lower overall survival rate because of anatomic constraints limiting functional resections rather than difference in biologic behavior and tumor histology. Given to their management difficulties, they must be treated in multi-disciplinary approach. Head and neck sarcomas are very rare tumors, accounting less than 1% from all malignancies of this region They represent a heterogeneous group of tumors with distinct prognostic features. Surgery remains the mainstay of treatment, despite the anatomic constraints, followed by radiation therapy
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