Variant Of Embryonal Rhabdomyosarcoma Research Articles

Sarcoma Botryoides a Rare Pediatric Pelvic Tumor: A Case Report and Literature Revue

Sarcoma botryoides, a variant of embryonal rhabdomyosarcoma, represents the predominant malignant tumor affecting the genitourinary (GU) system in children under 15 years of age. Among GU tract malignancies in this age group, the embryonal subtype of habdomyosarcoma is the most prevalent. In young and adolescent individuals, the cervix and uterus are affected; whereas in infants, vaginal lesions are more common. Imaging plays a crucial role not only in the initial diagnosis but also in long‑term follow‑up of genital rhabdomyosarcoma. Although the magnetic resonance imaging features of GU tract rhabdomyosarcomas have been escribed, there have been relatively few radiological reports in the literature regarding the botryoid variant. This article aims to illustrate and describe the imaging appearance of this rare tumor. We present a case of a one-year-old male who presented with a rapidly growing pelvic mass, crying and straining on micturition and difficulty in passing stool and urine. An abdomino-pelvic CT scan and MRI were performed. He had been examinated under anesthesia and biopsy. Histology confirmed sarcoma botryoides.

Case Report: Foley Catheter as Vaginal Stent in a Toddler With Vaginal Rhabdomyosarcoma

Sarcoma botryoides is a variant of embryonal rhabdomyosarcoma. This malignant tumor originates from muscle progenitor cells and although it is highly malignant, cure rates exceed 90%.1 While rhabdomyosarcoma is the most common soft tissue malignancy in children, vaginal presentation is rare.2 The average age of diagnosis of vaginal rhabdomyosarcoma is 21 months of age. Treatment consists of chemotherapy combined with local radiation to achieve local tumor control while preserving organ function.1 Sequelae of vaginal radiation therapy in this age group have not been well defined. However, vaginal stenosis is a known result of local radiation therapy in adult women treated for a variety of pelvic cancers. In adults, vaginal dilation is the primary method of prevention and treatment.3 An 18-month-old child presented with vaginal bleeding and was diagnosed with vaginal rhabdomyosarcoma. She underwent unilateral oophorectomy for fertility preservation prior to initiating a 40-week chemotherapy course. During weeks 24 to 26 of chemotherapy, she underwent vaginal brachytherapy radiation treatment. Six weeks following completion of radiation, vaginoscopy was performed with placement of non-latex 18 French (6mm) foley catheter to prevent vaginal stricture. When removed, vaginal patency was normal for her age with no vaginal erosion noted. Use of a soft, flexible, non-latex catheter provided a comfortable method of vaginal stricture prevention without need for vaginal dilation in a young child. Picture 1. Foley catheter vaginal stent can be visualized just proximal to the hymen. Three 5-0 prolene sutures were used to gently secure it in place. Picture 2. Following removal of the stent, normal vaginal patency and mucosa are visualized 1Kirsch CH, Goodman, M, Esiashvili N. Outcome of female pediatric patients diagnosed with genital tract rhabdomyosarcoma based on analysis of cases registered in SEER database between 1973 and 2006. Am J Clin Oncol (2012); 37:472Deligeoroglou E, Tsimaris P, Creatsa M, et al. Application of creatsas vaginoplasty after radical surgical treatment of sarcoma botryoides. J Pediatric Adolesc Gynecol 27 (2014); 93-95.3Miles T, Johnson N. Vaginal dilator therapy for women receiving pelvic radiotherapy. Cochrane Database of Systematic Reviews (2014), Issue 9. Art No: CD007291.

Embryonal Rhabdomyosarcoma

The aim of this retrospective study was to evaluate differences in treatment of embryonal rhabdomyosarcoma (RMS) of the uterus in 2 premenopausal women. We discuss adjuvant chemotherapy and use of ChemoFx Assay (Precision Therapeutics, Pittsburgh, PA) to guide choice of active chemotherapeutic agents. Two premenopausal patients were identified with a pathologic diagnosis of embryonal RMS of the uterus. Both met inclusion criteria for the study. A 21-year-old woman underwent a staging abdominal hysterectomy for a variant of embryonal RMS. Vincristine, actinomycin D, and cyclophosphamide were given adjunctively for a complete response. A 20-year-old woman underwent a diagnostic dilation and curettage revealing embryonal RMS. Initial treatment included an abdominal hysterectomy and nodal sampling. Presentation to a subsequent gynecologic oncologist 7 months later revealed recurrence. Carboplatin, doxorubicin, and paclitaxel provided a partial response. After a second surgical resection, ChemoFx Assay identified ifosfamide and mitomycin C as active agents and resulted in a complete response. Recommended treatment includes surgery and chemotherapy with possible radiation therapy if deemed necessary. The benefit of adding neoadjuvant or adjuvant chemotherapy and radiation therapy allows for a conservative surgical approach and improved survival. Choosing active chemotherapy agents can be aided by ChemoFx Assay. The chemotherapy most commonly used for treatment of embryonal RMS is a combination of vincristine, actinomycin D, and cyclophosphamide.

Primary rhabdomyosarcoma of the sacrum: a case report and review of the literature

We describe herein a rare case of primary rhabdomyosarcoma (RMS) occurring in the sacrum. A 16-year-old woman presented with a 2-month history of pain in bilateral buttocks and posterior thighs. Computed tomography showed a primary tumor with bone destruction in the 2nd sacral vertebra and invasion to the 1st to 3rd vertebrae and retroperitoneal space. Histological examination of the tumor showed proliferation of spindle-shaped cells intermingled with rhabdomyoblasts in a fascicular and storiform growth pattern. Tumor cells showed immunoreactivity for vimentin, desmin, muscle-specific actin, sarcomeric actin, alpha-smooth muscle actin and CD99, and partial immunoreactivity for myoD1, myf-4, myogenin and myoglobin. Reverse transcription polymerase chain reaction demonstrated expression of myoD1. On the basis of the aforementioned findings, a poorly differentiated spindle cell variant of embryonal RMS was diagnosed. The patient underwent combined therapy with chemotherapy and radiotherapy, but died 17 months after incisional biopsy. The present case is instructive in differential diagnosis of primary bone tumors, and the possibility of skeletal RMS needs to be considered.

Radiation-induced mandibular adult spindle cell rhabdomyosarcoma

Spindle cell rhabdomyosarcoma (RMS), a variant of embryonal RMS, is a rare tumour, especially in adults. Imaging techniques are used to evaluate the extent of the tumour whereas a biopsy is required to make the final diagnosis. In this article we describe a case of mandibular Spindle cell RMS in a previously irradiated field in a 56-year-old male, and discuss the clinicopathologic and imaging findings. Although the aetiology is still unclear, this case suggests that previous radiation therapy can be a causal factor.

Spindle cell rhabdomyosarcoma in adults. A case report and literature review

Spindle cell rhabdomyosarcoma, a recently described variant of embryonal rhabdomyosarcoma in children, carries a favorable prognosis when compared with other types of rhabdomyosarcoma. This tumor is rare in adults, and only four cases have been documented previously. The clinicopathological study of such a case occurring in the retroperitoneal space of a 53-year-old man is herein reported. The patient died of uncontrolled local recurrence and hepatic metastases seven months after diagnosis. Based on the analysis of the data of the five cases reported, including the present one, it can be stated that spindle cell rhabdomyosarcoma in adults is not associated with the favorable outcome observed in the pediatric population.

Spindle Cell Rhabdomyosarcoma (So-Called) in Adults

Spindle cell rhabdomyosarcoma (RMS) is a recently described variant of embryonal RMS that carries a relatively favorable prognosis when compared with other forms of RMS. To date, spindle cell RMS has been described only in children. The authors have identified two unusual cases occurring in adults using the following criteria: tumors composed mainly of fascicular, relatively monomorphic spindle-shaped cells that show unequivocal immunohistochemical and ultrastructural evidence of myogenic differentiation. The tumors were identified in a 38-year-old woman and a 56-year-old man, arising in the cheek and left hemidiaphragm, respectively. Both were treated with surgical resection and chemotherapy. The first patient died of uncontrolled local recurrence of her tumor at 27 months after diagnosis, and the second died of metastatic disease at 13 months follow-up. The tumors were composed mainly of fascicles of spindle cells with palely eosinophilic cytoplasm admixed diffusely with sparse polygonal, rounded, or strap-shaped rhabdomyoblasts with brightly eosinophilic cytoplasm and with cross-striations in the first case only. Immunostaining for muscle-related antigens showed staining for smooth-muscle actin (focal), pan-actin HHF-35, desmin, fast myosin, myoglobin, and MyoD1. Both cases were negative for S-100 protein. On electron microscopy, both cases showed neoplastic rhabdomyoblasts with clear-cut sarcomeric differentiation in many of the tumor cells. Spindle cell RMS poses special problems in differential diagnosis when arising in adults and should be distinguished from leiomyosarcoma, malignant peripheral nerve sheath tumor with heterologous rhabdomyoblastic differentiation (malignant Triton tumor), and fibrosarcoma. In view of the good prognosis afforded children with spindle cell RMS and in light of the chemoresponsive behavior of RMS in general, we feel that it is important to identify tumors that meet the criteria for spindle cell RMS occurring in the adult population. However, based on these two cases, it is possible that spindle cell RMS occurring in adults may not be associated with such a favorable outcome.

Congenital Spindle Cell Rhabdomyosarcoma of the Urinary Bladder

Spindle cell rhabdomyosarcoma is a recently described variant of embryonal rhabdomyosarcoma that carries a relatively favorable prognosis when compared with other types of rhabdomyosarcoma. We report the first case of a congenital spindle cell rhabdomyosarcoma in the urinary bladder of a newborn. The patient was diagnosed with obstructive uropathy and cystic kidneys in the antenatal period. The tumor was composed primarily of mature strap-shaped rhabdomyoblasts with cross-striations; it involved focally the full thickness of the bladder wall. No extension into adjacent organs or distant metastases were identified. The patient died of complications of urinary tract obstruction shortly after birth.