The Experts below are selected from a list of 282 Experts worldwide ranked by ideXlab platform
Athanasios Gouliamos - One of the best experts on this subject based on the ideXlab platform.
-
Embryonal Rhabdomyosarcoma of the uterine cervix
Clinical & Translational Oncology, 2009Co-Authors: Georgios V Koukourakis, Vassilios Kouloulias, Georgios Zacharias, Georgios Maravelis, Christos Papadimitriou, Kaliopi Platoni, Athanasios GouliamosAbstract:Introduction Embryonal Rhabdomyosarcoma (RMS) of the uterine cervix is a rare and extremely malignant entity. Generally, Embryonal RMS originating in the uterine cervix is usually diagnosed in adolescence. Before the introduction of effective adjuvant chemotherapy, the prognosis of these lesions was poor. We have treated a young woman suffering from this disease using a combination of surgery, chemotherapy and radiation therapy (RT) with excellent results. The medical community should keep in mind that Embryonal RMS of the uterine cervix, despite its malignancy and rarity, can be cured if adequate treatment is given.
Prasanta Kumar Gure - One of the best experts on this subject based on the ideXlab platform.
-
primary Embryonal Rhabdomyosarcoma of the anterior neck and thyroid
Laryngoscope, 2013Co-Authors: Mainak Dutta, Indranil Chatterjee, Prasanta Kumar GureAbstract:: Primary Embryonal Rhabdomyosarcoma of anterior neck involving the thyroid is extremely rare. This report is only the second of its kind that describes this form of nonorbital nonparameningeal Rhabdomyosarcoma in a 7-year-old boy and adds to a new, seldom-reported variant of Rhabdomyosarcoma in the head-neck region. The child presented with a huge anterior neck swelling that clinically resembled a thyroid mass. Computed tomography scan showed a heterogeneous mass in the anterior neck replacing the entire right lobe of thyroid. Fine-needle aspiration cytology was nondiagnostic. Right hemithyroidectomy with selective neck dissection suggested Embryonal Rhabdomyosarcoma by histopathology; the diagnosis was confirmed by positive reactions to desmin and myogenin. The child was subsequently treated with chemotherapy. Repeat chemotherapy with radiotherapy was required when recurrences were detected in the mediastinum and cervical lymph nodes at 13-month follow-up.
Prasanta Kumar Gure Ms - One of the best experts on this subject based on the ideXlab platform.
-
primary Embryonal Rhabdomyosarcoma of the anterior neck and thyroid
Laryngoscope, 2013Co-Authors: Mainak Dutta Ms, Soham Roy Md Facs Faap, Prasanta Kumar Gure MsAbstract:Primary Embryonal Rhabdomyosarcoma of anterior neck involving the thyroid is extremely rare. This report is only the second of its kind that describes this form of nonorbital nonparameningeal Rhabdomyosarcoma in a 7-year-old boy and adds to a new, seldom-reported variant of Rhabdomyosarcoma in the head-neck region. The child presented with a huge anterior neck swelling that clinically resembled a thyroid mass. Computed tomography scan showed a heterogeneous mass in the anterior neck replacing the entire right lobe of thyroid. Fine-needle aspiration cytology was nondiagnostic. Right hemithyroidectomy with selective neck dissection suggested Embryonal Rhabdomyosarcoma by histopathology; the diagnosis was confirmed by positive reactions to desmin and myogenin. The child was subsequently treated with chemotherapy. Repeat chemotherapy with radiotherapy was required when recurrences were detected in the mediastinum and cervical lymph nodes at 13-month follow-up. Laryngoscope, 123:2072–2076, 2013
Soham Roy Md Facs Faap - One of the best experts on this subject based on the ideXlab platform.
-
primary Embryonal Rhabdomyosarcoma of the anterior neck and thyroid
Laryngoscope, 2013Co-Authors: Mainak Dutta Ms, Soham Roy Md Facs Faap, Prasanta Kumar Gure MsAbstract:Primary Embryonal Rhabdomyosarcoma of anterior neck involving the thyroid is extremely rare. This report is only the second of its kind that describes this form of nonorbital nonparameningeal Rhabdomyosarcoma in a 7-year-old boy and adds to a new, seldom-reported variant of Rhabdomyosarcoma in the head-neck region. The child presented with a huge anterior neck swelling that clinically resembled a thyroid mass. Computed tomography scan showed a heterogeneous mass in the anterior neck replacing the entire right lobe of thyroid. Fine-needle aspiration cytology was nondiagnostic. Right hemithyroidectomy with selective neck dissection suggested Embryonal Rhabdomyosarcoma by histopathology; the diagnosis was confirmed by positive reactions to desmin and myogenin. The child was subsequently treated with chemotherapy. Repeat chemotherapy with radiotherapy was required when recurrences were detected in the mediastinum and cervical lymph nodes at 13-month follow-up. Laryngoscope, 123:2072–2076, 2013
Ashley D Hill - One of the best experts on this subject based on the ideXlab platform.
-
Embryonal Rhabdomyosarcoma of the uterine cervix a report of 14 cases and a discussion of its unusual clinicopathological associations
Modern Pathology, 2012Co-Authors: Louis P Dehner, Jason A Jarzembowski, Ashley D HillAbstract:Embryonal Rhabdomyosarcoma of the uterine cervix is an uncommon presentation of the most common soft-tissue sarcoma in the first decades of life. Unlike Embryonal Rhabdomyosarcoma in other anatomic sites, in which 70–80% of cases present before 9 years of age, the average age in our series of 14 cervical cases was 12.4 years (median, 13 years), with an age range of 9 months to 32 years at diagnosis. Of the 14 cases, 12 presented as a polyp at the cervical os; two patients had an infiltrative mass in the cervix without a botryoid polyp. The polyps measured 1.5–5 cm and all had the histopathological pattern of the sarcoma botryoides variant of Embryonal Rhabdomyosarcoma, with condensations of primitive and differentiated rhabdomyoblasts beneath the surface epithelium and around endocervical glands. Nodules of benign-appearing cartilage were present in the stroma of six cases (43%). One of the embyronal Rhabdomyosarcomas from the youngest patient, 9 months old, also had a distinctive microscopic focus of immature tubular profiles in a primitive stroma; these tubules expressed epithelial and neuroendocrine markers. Two patients had a pleuropulmonary blastoma, one diagnosed 9 years before the Embryonal Rhabdomyosarcoma of the cervix and the other recognized synchronously. This latter 9-year old had a DICER1 germline mutation. One patient presented with hirsutism and had a Sertoli–Leydig cell tumor, an incidentally detected cervical Embryonal Rhabdomyosarcoma, and nodular hyperplasia of the thyroid. Although a pleuropulmonary blastoma was not documented in the latter patient, ovarian sex-cord stromal tumors and nodular hyperplasia of the thyroid are manifestations of the pleuropulmonary blastoma family tumor and dysplasia syndrome (OMIM 601200). Embryonal Rhabdomyosarcoma of the cervix must be distinguished from other rare entities, including adenosarcoma, malignant mixed Mullerian tumor and low-grade stromal sarcoma, as the former has a better prognosis; 12 of our 14 patients remain disease-free following conservative surgery and chemotherapy. Our study suggests that cervical Embryonal Rhabdomyosarcoma may be another pathological manifestation in the spectrum of extrapulmonary pathology in the setting of pleuropulmonary blastoma.