Embryonal rhabdomyosarcoma

A fatal case of embryonal Rhabdomyosarcoma involving the ethmoid and orbital region is reported.     

Embryonal rhabdomyosarcoma of the prostate

We describe a case of embryonal rhabdomyosarcoma of the prostate in a 20-year-old man with a history of acute lymphatic leukemia at 6 years of age. He presented with gross hematuria and high fever. The level of prostate-specific antigen (PSA) was 27.9 ng/ml. Computed tomography revealed a suspected infectious cyst in the prostate. After antibiotic treatment was begun, the PSA level normalized. However, the patient later suffered bladder tamponade. A biopsy of the prostate revealed embryonal rhabdomyosarcoma. Chemotherapy with vincristine, actinomycin D, cyclophosphamide, and ifosfamide was begun according to the treatment protocol of the Japan Rhabdomyosarcoma Study Group. The tumor shrank, and partial remission was obtained after 1 course of chemotherapy. During treatment for acute lymphatic leukemia at the age of 6 years, the patient had been exposed to a cumulative radiation dose of 10 Gy across his entire body. It has been reported that 88% of postradiation sarcomas are KIT-positive, and we suspect that our patient suffered a postradiation sarcoma, because his tumor was KIT-positive. This is the first report of postradiation sarcoma manifesting as an embryonal rhabdomyosarcoma of the prostate.     

Embryonal rhabdomyosarcoma with intracranial extension

A case of embryonal rhabdomyosarcoma of the middle ear presenting with facial paralysis and a polypoidal aural mass who subsequently had convulsions due to intracranial extension is presented for its rarity.     

Embryonal rhabdomyosarcoma of the uterine cervix

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. Case  A case of a young woman aged 20, presenting with vaginal bleeding, is reported. The histological examination revealed embryonal RMS of uterine cervix. The patient was treated with a combination of surgery, chemotherapy and RT. A review in the literature, which is also presented, shows that the combined treatment of embryonal RMS using surgery and multidrug chemotherapy has significantly improved survival. Conclusion  Patients with favourable prognostic parameters, such as localised disease without deep myometrial invasion, single polyp and embryonal histologic subtype, can effectively be treated by surgery. Patients with unfavourable prognostic parameters seem to benefit from a multimodality approach including surgery, adjuvant chemotherapy and RT.     

Embryonal rhabdomyosarcoma of the ampulla of vater

The first case to appear in the world literature is described of an embryonal rhabdomyosarcoma in a child arising from the region of the ampulla of Vater and projecting into the lumen of the duodenum.     

Embryonal rhabdomyosarcoma of the tongue in old age

Rhabdomyosarcoma represents the most common soft tissue sarcoma of patients under 15 years of age, but it is definitely rare in subjects older than 45. A very rare case of embryonal type rhabdomyosarcoma of the tongue in a 65-year-old man is reported. The adult patient was treated with surgery and post-operative adjuvant radiotherapy and is disease-free at 48 months of follow-up. TUNEL analysis showed that apoptosis was present in 10% of the cells, thus suggesting that an high number of apoptotic cells may represent an indicator of good prognosis for this malignancy.     

Embryonal rhabdomyosarcoma of the middle ear and mastoid

A rare case of embryonal rhabdomyosarcoma of the middle ear and mastoid with facial paralysis is presented with brief review of literature. Embryonal rhabdomyosarcoma of the middle ear and mastoid is very rare. It is highly malignant tumour found in the paediatric age group. Soderserg (1933) reported the first case of embryonal Rhabdomyosarcoma of the middle ear. Stobbe and Drageon (1959) described 15 cases of tumour occuring in the head and neck, out of which two were in the middle ear and the mastoid process. Deutsch and Felder (1974) reviewed the world literature up to 1973 and could collect only 22 cases including 5 of their own. According to Maurer et al (1977) rhabdomyosarcoma originating in the head and neck comprise 36% of all cases of rhabdomyosarcoma. A rare case of Embryonal Rhabdomyosarcoma of the middle ear and mastoid with facial paralysis is being reported with a brief review of literature.     

Embryonal rhabdomyosarcoma of the mastoid in an infant

A case of embryonal rhabdomyosarcoma of the mastoid in a six weeks old infant is reported because of rarity and unusually early onset of the disease.     

Embryonal rhabdomyosarcoma of prostate in an adult--a diagnostic dilemma

Embryonal rhabdomyosarcoma of the prostate is a rare. Highly malignant tumour. The median age of occurrence is five years, but sporadic cases have been reported in adults' To the best of our knowledge, till date, fewer than ten cases have been reported of which only two are above the age of sixty years. We report a case of embryonal rhabdomyosarcoma of prostate in a patient more than sixty years of age. If one is not aware of this entity, one can make a mistake in the diagnosis as well as treatment.     

Embryonal rhabdomyosarcoma of the biliary tree mimicking a choledochal cyst

Embryonal rhabdomyosarcoma (ERMS) of biliary tree is a rare type of mesenchymal neoplasm diagnosed at surgery or by preoperative liver biopsy. We present a one year eight months old child who mimicked a choledochal cyst and was eventually treated with surgery, chemotherapy with IRS IV protocol and adjuvant postoperative radiotherapy to surgical bed with 6 MV photons to a dose of 5040 cGy in 28 fractions.     

Embryonal cancers in Europe

Embryonal cancers are a heterogeneous group of rare cancers which mainly occur in children and adolescents. The aim of the present study was to estimate the burden (incidence, prevalence, survival and proportion of cured) for the principal embryonal cancers in Europe (EU27), using population-based data from cancer registries (CRs) participating in RARECARE. We identified 3322 cases diagnosed from 1995 to 2002 (latest period for which data are available): 44% neuroblastoma, 35% nephroblastoma, 13% retinoblastoma and 6% hepatoblastoma. Very few cases of pulmonary blastoma (43 cases) and pancreatoblastoma (seven cases) were diagnosed. About 2000 new embryonal cancers were estimated every year in EU27, for an annual incidence rate of 4 per million (1.8 neuroblastoma, 1.4 nephroblastoma, and 0.5 retinoblastoma); 91% of cases occurred in patients under 15 years. Five-year relative survival for all embryonal cancers was 80% (99% retinoblastoma, 90% nephroblastoma, 71% hepatoblastoma and 68% neuroblastoma). Overall survival was lower in adolescents and adults than in those under 15 years. The cure rate was estimated at 80%. Slightly less than 40,000 persons were estimated alive in EU27 with a diagnosis of embryonal cancer in 2008. Nephroblastoma was the most prevalent (18,150 cases in EU27), followed by neuroblastoma (12,100), retinoblastoma (5200), hepatoblastoma (2700) and pulmonary blastoma (614). This is the first study to delineate the embryonal cancer burden in Europe by age, sex and European region. Survival/cure rate is generally high, but there are considerable gaps in our understanding of the natural histories of these rare diseases particularly in adults.     

Embryonal carcinoma of the testis

Long-term survival rates were correlated with selected clinical features in 479 patients with embryonal carcinoma of the testis and 33 patients with endodermal sinus tumor (infantile embryonal carcinoma, yolk sac tumor). In the period 1977 to 1982 embryonal carcinoma accounted for 26.8% of newly diagnosed germ cell tumors and 43% of nonseminomatous germ cell tumors entered in the Centralized Cancer Patient Data System. Among patients with embryonal carcinoma, over 80% were diagnosed in the 15-to-34 year age group. Seventy-four percent of the patients had metastatic disease at the time of diagnosis, and 50% of these had distant metastases, attesting to the aggressiveness of embryonal carcinoma and its tendency to early hematogenous spread. Despite the highly malignant nature of the tumor, the overall 5-year survival rate with treatments used was an excellent, 88%. Survival was correlated with the extent of disease at the time of diagnosis; the 5-year actuarial survival rates for patients with localized, regional, and distant disease were 98%, 96%, and 74%, respectively. Endodermal sinus tumor was uncommon (1.8% of all testicular germ cell tumors), occurred predominantly in the younger age group (0-24 years), and in 50% of the cases was localized to the testis. The survival rate for the 33 patients with this form of tumor was slightly worse than for the "adult form" of embryonal carcinoma. The authors conclude that survival of patients with embryonal carcinoma has greatly improved over the last decade as a result of improved methods for early detection of metastatic deposits and the effectiveness of newer chemotherapies in the treatment of disseminated disease.     

Embryonal Rhabdomyosarcoma of tympano-mastoid region

A rare case of Embryonal Rhabdomyosarcoma of the tympanomastoid region with paralysis of VIth and VIIth cranial nerve and development of ipsilateral loss of vision is reported. The case was first clinically diagnosed as a complicated case of C.S.O.M. with petrositis and facial palsy and was operated upon. Histopathological examination in the postoperative period proved it to be a case of Embryonal Rhabdomyosarcoma.