Adult Embryonal Rhabdomyosarcoma Research Articles

18F-fluorodeoxyglucose Positron Emission Tomography/Computed Tomography and 68Ga-prostate-specific Membrane Antigen Positron Emission Tomography/Computed Tomography Imaging in the Evaluation of Rare Entity Adult Embryonal Rhabdomyosarcoma of Prostate.

A 21-year-old male with embryonal rhabdomyosarcoma of the prostate was referred for 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) and 68Ga-prostate-specific membrane antigen (PSMA) PET/CT for initial disease staging. The PET scans revealed hypermetabolic and PSMA expressing lobulated mass involving both lobes of the prostate and weakly metabolic and PSMA expressing few bilateral pararectal and external iliac nodes, multiple bilateral lung nodules scattered over the lung parenchyma and multiple bone marrow lesions in both axial and appendicular skeleton. Magnetic resonance imaging prostate showed gross prostatomegaly with large lobulated T2 hyperintense heterogeneously enhancing mass lesion showing restricted diffusion, involving both lobes of the prostate with extraprostatic spread along anterior, posterior, and left lateral margins with evidence of lymph nodal and osseous metastases. The demonstration of increased uptake of 18F-FDG and 68Ga-PSMA in the primary as well as bilateral pararectal and external iliac nodes, multiple bilateral lung nodules, and multiple bone marrow lesions in both axial and appendicular skeleton indicates a potential role of 18F-FDG PET/CT and 68Ga-PSMA PET/CT in disease staging in this rare aggressive tumor of the prostate.

Primary paratesticular embryonal rhabdomyosarcoma - An unusual presentation.

Paratesticular embryonal rhabdomyosarcoma (RMS) is a very rare and aggressive mesenchymal tumor. It is usually seen in children and adolescents presenting as a painless intrascrotal mass, localized in the paratesticular region. Hereby, we report two cases of paratesticular embryonal RMS in adults. One case was clinically suspected to be a testicular abscess, whereas the other presented with testicular swelling and lung metastasis. Localized forms have a good prognosis, whereas tumors presenting with metastases show a poor outcome. A treatment based on surgery and chemotherapy yields good results. Sperm cryopreservation and endocrine follow-up improve the overall survival and quality of life of these patients.

An uncommon perineal embryonal rhabdomyosarcoma in adult: A case report

Embryonal rhabdomyosarcoma (ERMS) is a major subtype of rhabdomyosarcoma, mainly affect children. There is seldom report for perineal ERMS in adults, since its rare location and the age. A 20-year old male adult was admitted due to the perineal mass. Diagnosis by histopathological examination of the biopsy sample was ERMS. Magnetic resonance imaging showed the tumor was found in the perineal region, with metastasis to pelvic cavity, right testis, lymph nodes and bone. The patient received Isophosphamide and Epirubicin for 4 cycles, followed by Irinotecan and Vindesine Sulfate for 2 cycles, then cisplatin, Dacarbazine and Apatinib for 3 cycles. The patient showed no response to chemotherapy. Perineal ERMS in adults is very rare. There is still no standard therapy for adult ERMS. Personalized therapy might be promising treatment for each individual.

Embryonal Rhabdomyosarcoma: A Tale of Two Cases with Unusual Presentation

Rhabdomyosarcoma (RMS) is a rare soft tissue tumour, accounting for 3% of all childhood cancers. RMS can arise virtually anywhere in the body as it originates in embryonal mesenchyme. Embryonal Rhabdomyosarcoma (ERMS) and Alveolar Rhabdomyosarcoma (ARMS) are the most prevalent subtypes of RMS but arise through diverse biological mechanisms. RMS generally presents as an expanding mass that tends to be very painful and causes symptoms related to the compression of structures present nearby. Metastases of such tumours are common and can occur in bone, lungs and other organs leading to pain, difficulty with respiration, pleural effusion, anaemia, thrombocytopenia, and neutropenia. In this article, two unusual presentations of ERMS have been reported. First case is of a 28-year-old adult male who presented in surgical Out Patient Department (OPD) with recurrent episodes of painful acute urinary retention since one year. Another case was of a six-year-old girl presenting with aural fullness and serosanginous discharge from right ear, ear pain and decreased hearing since two years. Since five year survival rate of such tumour is less than 30%, therefore, an awareness of the typical signs and symptoms, radiological features, histomorphological features in a case of paediatric and adult ERMS can help a pathologist to consider this tumour in the differential diagnoses, even at unusual sites.

Adult embryonal rhabdomyosarcoma of the nose and paranasal sinuses misdiagnosed as an orbital cellulitis: a case report

 Rhabdomyosarcoma is a primary malignant tumor, which develops from normal skeletal muscle tissue. It´s a tumor quite common in children and exceptional in adults. This tumor constitutes a diagnostic and therapeutic emergency based on multidisciplinary management. We present the case of a 22-year-old woman admitted for left orbital swelling and purulent rhinorrhea initially considered as orbital cellulitis. The unsatisfactory outcome under oral and topical antibiotic therapy justified a biopsy of the lesion revealing embryonal rhabdomyosarcoma. A treatment combining chemotherapy and radiotherapy was immediately indicated and started. This protocol is increasingly accepted because of its low morbidity. Although facial rhabdomyosarcoma is relatively rare, it should be considered as a potential differential diagnosis of nasal and paranasal sinus tumors not only in children, but also in adults. The originality of our case is due to the young age of our patient and the histological type of the tumor.

Giant intrascrotal embryonal rhabdomyosarcoma in an adult: a case report and review of the\xa0literature

BackgroundIntrascrotal embryonal rhabdomyosarcoma in adults is a rare tumor with high aggression and a poor prognosis. We report our patient’s case and review the relevant literature to improve the understanding of this rare disease.Case presentationA 21-year-old Han Chinese man presented to our hospital with a right intrascrotal mass of 1 year’s duration. His physical examination revealed an enlarged right scrotum containing a huge tender mass measuring about 10 × 7 cm. Ordinary and contrast-enhanced ultrasonography showed a solid mass in the right scrotum, which was suspected to be a malignant tumor. An abdominopelvic computed tomographic scan revealed metastases in the retroperitoneal lymph nodes. The patient was diagnosed with malignant testicular tumor and underwent a right radical orchiectomy by an inguinal approach. Postoperative pathological examination suggested an intrascrotal embryonal rhabdomyosarcoma.ConclusionsIntrascrotal embryonal rhabdomyosarcoma is a rare but highly aggressive tumor. Clinical and imaging manifestations of this tumor are nonspecific, so the definitive diagnosis depends on postoperative pathology and immunohistochemistry. Early suspicion, radical orchiectomy, accurate pathologic diagnosis, and adjuvant chemotherapy and/or radiotherapy are the keys to optimal prognosis.

Failed organ preservation strategy for adult laryngeal embryonal rhabdomyosarcoma

PurposeTo present a case of embryonal rhabdomyosarcoma of the intrinsic laryngeal musculature and discuss the treatment of this rare tumor. Methods and materialsA 45year-old male presented with an eleven month history of hoarseness. A mass of the posterior glottis was noted on fiberoptic laryngoscopy. Computed tomography indicated a 1.5×2.5cm laryngeal mass without cartilage involvement. Direct laryngoscopy and biopsy were consistent with embryonal rhabdomyosarcoma involving the interarytenoid muscle. A multidisciplinary tumor board recommended multimodality therapy including total laryngectomy. His case was reviewed along with the available English language literature. ResultsThe patient refused surgery, and he qualified for a pediatric rhabdomyosarcoma treatment protocol with induction chemotherapy followed by combined chemoradiation. There was no noted response and the patient was taken off protocol to increase the radiation dose without chemotherapy. Fifteen weeks following radiation, a repeat biopsy revealed viable tumor. The patient elected further experimental chemotherapy at an outside hospital. The patient currently is alive at 34months post-treatment with suspected persistent disease and continues to refuse surgery. ConclusionsEmbryonal rhabdomyosarcoma involving the larynx is an extremely rare tumor usually seen in children. There have been few previous adult cases reported and treatment options are not well described. We present a case of chemoradiation failure in an adult with embryonal rhabdomyosarcoma refusing surgical intervention. Although pediatric tumors can be effectively treated with organ preservation strategies, adult tumors may have a poorer response. Based upon our experience and existing literature regarding adult embryonal rhabdomyosarcoma of the larynx, multimodality therapy including surgical resection should be the treatment of choice.

Prostate embryonal rhabdomyosarcoma in adults: Case report and review of literature

IntroductionProstate embryonal rhabdomyosarcoma (ERMS) is a common tumour in infants and children, with a median occurrence age of 5 years, but it is rare in adults. It is characterized by a high degree of malignancy, both local rapid growth with formation of large pelvic masses, often leading to renal failure due to urethral obstruction, and systemic spread, commonly to the lungs, liver and bone. Several therapeutic approaches have been employed in the effort to treat prostate ERMS, but all of them have failed to gain a significant survival benefit in adult patients. Case reportWe report on a case of a stage IV prostate ERMS, approached with combined-modality treatment, with the administration of 5 courses of doxorubicin, ifosfamide and 2-mercaptoethane sulfonate sodium (mesna), and, subsequent radiotherapy to the prostatic bed (60Gy/30fxs). The patient remained free of progression of disease for about 1 year to finally experience a systemic relapse with multiple lung metastases and pleural effusion. The patient died for metastatic disease 27 months following the initial diagnosis. ConclusionWhile it remains questionable which therapeutic approach for prostate ERMS in adults is the most appropriate, our report demonstrates that a chemo-radiation combined treatment can control the prostate disease, reducing the symptoms and improving the quality of life of these patients, for the most part destined to die for systemic progression of disease.

Embryonal Rhabdomyosarcoma Occurring on Mandibular Gingiva in an Adult

An embryonal rhabdomyosarcoma (ERMS) is a primitive, malignant, soft tissue sarcoma that recapitulates the phenotypic and biological features of embryonic skeletal muscles. Occurrence of intraoral ERMS in adults is extremely rare. This unique case report highlights the clinical, radiographic, histopathological and immunohistochemical findings of an intraoral ERMS.

Primary Renal Embryonal Rhabdomyosarcoma in Adults: A Case Report and Review of the Literature

Adult renal rhabdomyosarcoma is a rare subtype of renal sarcoma. We present a case of a renal mass treated with radical nephrectomy that subsequently was shown to be renal rhabdomyosarcoma. We discuss the clinical presentation, imaging findings, and histology for this case and review the available literature.

Embryonal rhabdomyosarcoma of the head and neck in an adult

This report describes an exceedingly rare case of adult embryonal rhabdomyosarcoma arising in the head and neck, which was initially diagnosed as a primary unknown cancer. The patient underwent a radical neck dissection with the total excision of the tumor, which was diagnosed as embryonal rhabdomyosarcoma. The patient received intensive adjuvant chemotherapy, and is still alive with no signs of recurrence or metastasis. This case report demonstrates not only the rarity of this neoplasm but the importance of the differential diagnosis for planning the therapeutic strategy.

Ifosfamide, Doxorubicin and Dacarbazine Chemotherapy, and Adjunctive Laparoscopic Cystoprostatectomy and External Beam Radiotherapy for Prostatic Embryonal Rhabdomyosarcoma

No AccessJournal of UrologyCLINICAL UROLOGY: Case Reports1 Dec 2002Ifosfamide, Doxorubicin and Dacarbazine Chemotherapy, and Adjunctive Laparoscopic Cystoprostatectomy and External Beam Radiotherapy for Prostatic Embryonal Rhabdomyosarcomais corrected byErrata H. CHALCHAL, T.H.P. TAI, E. TSE, E.C. ALPORT, and M. SALIM H. CHALCHALH. CHALCHAL More articles by this author , T.H.P. TAIT.H.P. TAI More articles by this author , E. TSEE. TSE More articles by this author , E.C. ALPORTE.C. ALPORT More articles by this author , and M. SALIMM. SALIM More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(05)64195-XAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "Ifosfamide, Doxorubicin and Dacarbazine Chemotherapy, and Adjunctive Laparoscopic Cystoprostatectomy and External Beam Radiotherapy for Prostatic Embryonal Rhabdomyosarcoma." The Journal of Urology, 168(6), p. 2545 References 1 : Prostate embryonal rhabdomyosarcoma in adults. A clinicopathologic review. Cancer1992; 69: 755. Crossref, Medline, Google Scholar 2 : The Intergroup Rhabdomyosarcoma Study. I. A final report. Cancer1988; 61: 209. Crossref, Medline, Google Scholar From the Allan Blair Cancer Centre, University of Saskatchewan, Regina, Saskatchewan, Canada© 2002 by American Urological Association, Inc.FiguresReferencesRelatedDetailsCited by Kieran K and Shnorhavorian M (2016) Current standards of care in bladder and prostate rhabdomyosarcomaUrologic Oncology: Seminars and Original Investigations, 10.1016/j.urolonc.2015.12.012, VOL. 34, NO. 2, (93-102), Online publication date: 1-Feb-2016. Related articlesJournal of Urology9 Nov 2018Errata Volume 168Issue 6December 2002Page: 2545 Advertisement Copyright & Permissions© 2002 by American Urological Association, Inc.Keywordsprostatic neoplasmsradiotherapyprostatectomydrug therapy, combinationrhabdomyosarcoma, embryonalMetricsAuthor Information H. CHALCHAL More articles by this author T.H.P. TAI More articles by this author E. TSE More articles by this author E.C. ALPORT More articles by this author M. SALIM More articles by this author Expand All Advertisement PDF downloadLoading ...

Prostatic embryonal rhabdomyosarcoma in adults. A clinicopathologic review

Embryonal rhabdomyosarcoma of the prostate is a rare, highly malignant tumor that occurs predominantly in male infants and children, in whom it is the most common prostatic sarcoma. Six cases occurring in adults have been published, and the authors report three additional cases. The natural history is characterized by rapid growth, with the typical formation of large pelvic or abdominal masses, often leading renal failure due to bilateral ureteric obstruction. The tumor eventually disseminates widely, mainly to the lungs, bone, liver, and serosal surfaces, and unlike most other sarcomas, regional lymph node metastases are common. Combined modality therapy has resulted in marked improvement in survival rates and reduced surgical morbidity for children with these tumors. However, in adults the prognosis remains poor, with all patients dying of disseminated disease within 16 months of histologic diagnosis (mean survival, 8 months).

Adult Embryonal Rhabdomyosarcoma of the Vagina Complicating Pregnancy: A Case Report and Review of the Literature

Embryonal rhabdomyosarcoma of the vagina in adults per se is uncommon and pregnancy complicated by such tumors is extremely rare. We wish to report one case of vaginal embryonal rhabdomyosarcoma, diagnosed at the age of 23 years who was carrying at the time of initial presentation. The problem of management of rhabdomyosarcoma in adults, particularly when they are associated with pregnancy is discussed. We advocate the use of multiagent chemotherapy and pelvic radiotherapy in its overall management.

Embryonal rhabdomyosarcoma in adults.

A series of 54 cases of embryonal rhabdomyosarcomas in adults were studied. The neoplasms were classified according to anatomic site, size, histologic pattern, age, and sex of the patients and modality of treatment. The major anatomic sites were the extremities (21 cases), genitourinary organs (13 cases) and the trunk (nine cases). The overall five-year survival rate was 21% but 79% of the patients were dead at an average time of 17 months after the primary diagnosis was made. On the other hand, 80% of the patients with Stage II disease (tumor size less than 5 cm) survived five years. In general, patients who were treated with surgery only or surgery and radiation or chemotherapy did much better than patients treated with chemotherapy and/or radiation therapy only. When the rate of recurrences, metastasis and time of death due to spread of neoplasm was compared in cases diagnosed between 1950-1969 and 1970-1978, patients in the last nine years had fewer recurrences, less metastases and better survival than those who had their neoplasm diagnosed and treated between 1950 and 1969.