Context:Clear cell Hidradenocarcinoma is a rare carcinoma arising from sweat glands. It is an aggressive tumor that most metastasizes to regional lymph nodes and distant viscera; surgery with safe margins is the mainstay of treatment.Case Report:We report a case of 68-year-old woman who presented with an invasive clear cell hidradenocarcinoma situated in the left parotid area which recurred 5 months after surgery, this recurrence was managed successfully by high-dose irradiation of the tumor bed (66 Gy) and regional lymphatic chains (50 Gy), after a follow-up of more than 15 months, the patient is in good local control without significant toxicity.Conclusion:Post operative radiotherapy allows better local control and should be mandatory when histological features predictive of recurrence are present: positive margins, histology poorly differentiated, perineural invasion, vascular and lymphatic invasion, lymph node involvement, and extracapsular spread.
IntroductionEmbryonal rhabdomyosarcoma of the female genital tract is rare in the cervix. It has been mainly discussed in the context of individual case studies. It tends to occur in children and young women. Treatment ranges from radical surgery to conservative surgery, followed by chemotherapy.Case presentationA 16-year-old Moroccan adolescent girl presented to our center with a protruding mass from her vaginal introitus, as a polyp of 6cm. An examination revealed a polyp within her vagina, thought to be arising from her cervix and a polypectomy was performed. Microscopic findings are consistent with an embryonal rhabdomyosarcoma (botryoide type). A computed tomography of her thorax, abdomen and pelvis were performed and residual disease was found as a mass located at her cervix, which measured approximately 4.5cm in its widest dimensions, without evidence of metastatic disease. Due to the fact that she is young, after discussions in a multidisciplinary meeting, she was subsequently treated with four cycles of multi-agent chemotherapy. Two cycles of chemotherapy and radiotherapy were administered due to the lack of response, but she presented vaginal bleeding with persistence of the same mass in computed tomography. Hence a total interadnexal hysterectomy was made. A histologic examination found residual embryonal rhabdomyosarcoma (botryoide type) located in all her cervix and she is currently under chemotherapy.ConclusionsThe presence of a cervical polyp in an adolescent is a gynecologic oddity and must necessarily be examined histologically because it might be a rhabdomyosarcoma. This is extremely important because diagnosis at an early stage of the disease is a highly favorable prognostic factor that allows “fertility-sparing surgery” for these young patients.
Fibrosarcoma arising in the sinonasal cavities are very rare. By the time of clinical diagnosis, they are usually advanced. Lack of adequate surgical margins predisposes these patients to tumor recurrences. Most common sites are the extremities, with only one percent of fibrosarcoma arising in the head and neck area. The imaging features of these tumors reflect their aggressive behavior. We report the case of a maxillary sinus fibrosarcoma in a 16 year-old male patient. Neoadjuvant chemotherapy, medial maxillectomy, adjuvant chemotherapy and subsequent external irradiation was perfomed.
IntroductionAdenoid cystic carcinoma is a malignant epithelial neoplasm derived from the salivary glands. Primary adenoid cystic carcinoma of the cervix is extremely rare, accounting for less than 1% of all cervical carcinomas. In this paper we report a case of primary adenoid cystic carcinoma and a review of the related literature.Case presentationA 68 year-old woman was admitted with signs and symptoms suggestive of a cervical cancer. The radiological and pathological investigations confirmed the diagnostic of primary adenoid cystic carcinoma of the cervix at Stage IIIB according to the International Federation of Gynaecology and Obstetrics classification. The patient was managed successfully by concurrent chemo-radiotherapy.ConclusionThe optimal management of adenoid cystic carcinoma cannot be established for certain. From our case and from the literature, it appears that combined treatment (surgery, radiotherapy, and chemotherapy) is necessary for achieving a long-term remission. Concurrent chemo-radiotherapy appears to be a logical option for locally advanced disease.
IntroductionPeriductal stromal sarcoma is an extremely rare malignant fibroepithelial tumor of the breast which is characterized by its biphasic histology with benign ductal elements and a sarcomatous stroma made of spindle cells and lacking phyllodes architecture. Its therapeutic management is based on wide surgery with free margins. Adjuvant therapies are not needed. Periductal stromal sarcoma may evolve into a phyllodes tumor with time, as well as a specific soft-tissue sarcoma. To the best of our knowledge, this tumor has never been described in a child.Case presentationA 14-year-old Arabic boy was presented to our hospital one year ago with a nodule of the right breast that was gradually increasing in size without signs of inflammation. The histological examination after lumpectomy revealed a periductal stromal sarcoma with free surgical margins. No adjuvant treatment was given. At 50 months of close follow-up, no recurrence was observed.ConclusionPeriductal stromal sarcoma in a child is a very rare disease which has the same indolent behavior as it does in adults. Therefore, close follow-up is required.
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