Paratesticular rhabdomyosarcoma in a low-resource setting: a case report of an aggressive disease amidst systemic healthcare limitations

Para-testicular rhabdomyosarcoma (PTRMS) is a rare tumor, and it accounts for 7% of all rhabdomyosarcoma tumors. Among all the rhabdomyosarcoma (RMS) types, the spindle cell RMS is extremely rare. The present study describes a case of a para-testicular spindle cell RMS that was treated with a radical inguinal orchiectomy (RIO) and right scrotal resection. A 17-year-old male patient presented with a half-year history of a rapidly growing, painless, right scrotal mass. His CT of the pelvic cavity showed a mixed-density mass in the right scrotum, and the maximum cross-sectional area was approximately 76.5mm × 64.5mm. An X-ray of the chest demonstrated no evidence of metastasis, and a local surgical excision was performed subsequently. The histopathological and immunohistochemical examination confirmed the final diagnosis of spindle cell RMS. As a newly diagnosed case, strict and regular follow-up is needed. This article focuses on the importance of prompt recognition, diagnosis, pathological features, and appropriate management of para-testicular spindle cell RMS.

Alveolar rhabdomyosarcoma of the tunica vaginalis presenting as a tender hydrocele

Testicular and paratesticular rhabdomyosarcomas (RMSs) in children are uncommon tumors. Histologically though embryonal RMS is common, the spindle cell variant is considered rare. Paratesticular RMS presents in children and adolescents with a unilateral, painless scrotal swelling or mass above the testis. We report the case of a 15-year-old boy, with a left paratesticular mass who underwent high inguinal orchidectomy. Histopathological examination of the specimen demonstrated spindle cell RMS (SC-RMS). Because of its morphological resemblance to spindle cell neoplasms such as leiomyosarcomas and fibrosarcomas, SC-RMS may pose diagnostic difficulties for the pathologist. This problem can be overcome by a careful search for rhabdomyoblasts in sections and immunohistochemistry for myogenin. We are reporting this case as paratesticular RMS itself is uncommon, and the spindle cell variant of embryonal RMS is all the more rare. There are lacunae in our knowledge about their presentation, diagnosis, response to treatment, and cure.

Paratesticular rhabdomyosarcoma (RMS) is a rare malignancy, representing ~3% of all pediatric soft tissue tumors. The spindle cell subtype, a variant of embryonal RMS, is particularly uncommon but is typically associated with a favorable prognosis. We report the case of a 12-year-old boy who presented with a painless, progressively enlarging right inguinoscrotal mass. Imaging studies revealed a heterogeneous lesion without distant metastasis, and tumor markers were within normal limits. The patient underwent radical orchiectomy, and histopathology confirmed spindle cell RMS, with immunohistochemical positivity for myogenin and desmin. After completing treatment with the Children’s Oncology Group (COG)-ARST0531 Vincristine, Actinomycin D, and Cyclophosphamide protocol, a retroperitoneal lymph node recurrence was detected 1 year later. Management consisted of surgical resection and escalated chemotherapy per the COG-ARST0431 protocol, with subsequent imaging showing no evidence of disease. This case demonstrates that despite a favorable histology, paratesticular spindle cell RMS carries a risk of late, regional recurrence, underscoring the necessity of vigilant long-term surveillance. We also performed a systematic literature review to contextualize our findings, focusing on clinical presentation, therapeutic strategies, and outcomes.

Testicular tumors in children

Paratesticular rhabdomyosarcoma in adolescents: Challenging diagnosis and poor prognosis of a rare entity.

BackgroundParatesticular rhabdomyosarcoma is a sporadic tumor arising from the para-testicular structures, and is often diagnosed in children and adolescents.ObjectiveTo highlight the aggressive nature of rhabdomyosarcoma and the feasibility of inguinal orchidectomy for a giant locally advanced paratesticular rhabdomyosarcoma. This case is unique because it is one of the few reported cases of adult paratesticular rhabdomyosarcoma. It also discusses the surgical and oncological management of paratesticular rhabdomyosarcoma.Case presentationAn 18-year-old young adult presented to the emergency department of Federal Teaching Hospital Gombe with a 6-month history of right hemi-scrotal mass. He was chronically ill-looking and pale with mobile and non-tender bilateral superficial inguinal lymphadenopathy. There was a giant and tender right hemiscrotal swelling extending to the groin which was firm to hard in consistency with an area of skin ulceration. A malignant right testicular tumor was diagnosed. Serum alpha-feto protein, beta-human chorionic gonadotropin, and lactate dehydrogenase were within normal range. The abdominopelvic ultrasound scan and chest x-ray were normal. Scrotal ultrasound scan showed a huge lobulated heterogenous hypoechoic right intratesticular mass extending to the inguinal region with focal areas of increased echogenicity with no colour flow. He underwent a right inguinal orchidectomy. A huge right testicular tumor with solid and cystic components measuring 26 cm × 10 cm × 10 cm with the overlying ulcerated skin was resected en-bloc. The histology was consistent with an embryonal rhabdomyosarcoma. The patient recovered uneventfully and was referred for adjuvant therapy.ConclusionEmbryonal paratesticular rhabdomyosarcoma is rare, especially in adults and its diagnosis is mostly established following histology. This is because ultrasound findings are often non-specific and testicular tumor markers are not elevated. Inguinal orchidectomy is feasible even in the presence of a giant testicular tumor. This provides local tumor control, reduces the need for repeated blood transfusions, and provides specimens for definitive diagnosis. Adult patients have a worse prognosis with less than 30% overall 5-year survival and therefore, long-term follow-up is needed.

Paratesticular rhabdomyosarcoma (RMS) is a rare pediatric malignancy often misdiagnosed as benign conditions like epididymitis. We report a 4-year-old boy with paratesticular RMS and retroperitoneal metastasis, initially mistaken for epididymitis. The diagnostic delay resulted in disease progression, necessitating radical inguinal orchiectomy, hemiscrotectomy, and complete multimodal therapy. Literature review identified nine similar misdiagnosed pediatric cases due to atypical presentation. Despite a generally favorable prognosis, early diagnosis, radical orchiectomy, and multidisciplinary management are essential to reduce morbidity and improve survival outcomes in children with paratesticular RMS. Clinicians should consider RMS in children with persistent or atypical scrotal masses to avoid treatment delays.

Paratesticular rhabdomyosarcoma: a rare case report from Syria.

Objective To explore the clinical characters and treatment of paratesticular rhabdomyosarcoma (RMS) in children. Methods The clinical data of a child patient with paratesticular RMS was retrospectively analyzed and the related literatures were reviewed. Results The patient presented with a progressively enlarging mass in left scrotum. Biochemical test, immunologic test, tumor marker showed no abnormalities. Ultrasound demonstrated a solid lesion in the left scrotum with rich blood supply signals inside. Enhanced magnetic resonance showed the same result as ultrasound. Radical left testicular resection was performed. Pathological diagnosis was paratesticular RMS with positive result of Desmin and Myogenin. A painless mass was found in the left scrotum on the third day after operation, then left scrotum resection was performed. VAC chemotherapy was used after operation. Follow-up for 8 months, the patient survived with tumor free. Conclusions The paratesticular RMS can present as a mass in scrotum and is easily considered to be carcinoma of testis because of its location. Correct diagnosis and reasonable treatments can be made by pathologic examination after radical resection. Key words: Child; Rhabdomyosarcoma; Paratesticular; Diagnosis

Retroperitoneal lymph node dissection (RPLND) is recommended in children 10 years or older with paratesticular rhabdomyosarcoma (PTRMS). Primary tumors >5 cm are an additional risk factor for disease recurrence in the retroperitoneum. We report our experience with laparoscopic RPLND (LRPLND) in high-risk pediatric patients with PTRMS. Three patients, mean age 13.6 years (range 10-16 yrs), underwent modified template LRPLND after radical orchiectomy for preoperative rhabdomyosarcoma stage T(1a)N(0)M(0), T(1b)N(0)M(0), and T(2b)N(0)M(0), respectively. Primary paratesticular masses measured a mean 7.5 cm (range 4-10 cm). LRPLND was performed a mean of 8.6 days (range 7-12 d) after radical orchiectomy using four trocars that were placed equidistant in the midline. Average operative time was 382 minutes (range 245-656 minutes). Mean estimated blood loss was 53 mL (range 10-75 mL), and mean postoperative hospital stay was 2.5 days (range 2-3 d). There were no postoperative complications. Retroperitoneal nodes had negative findings for microscopic disease in two patients and positive findings in one patient. All patients received adjuvant chemotherapy with vincristine, actinomycin, and cyclophosphamide. LRPLND for high-risk pediatric patients with PTRMS is a safe diagnostic and therapeutic procedure with the benefit of rapid convalescence, enabling early commencement of adjuvant chemotherapy.

Impact of Hemiscrotectomy on Outcome of Patients with Embryonal Paratesticular Rhabdomyosarcoma: Results from the Cooperative Soft Tissue Sarcoma Group Studies CWS-86, 91, 96 and 2002P

To ascertain whether alveolar histology retains its adverse prognostic role in the subset of paratesticular rhabdomyosarcoma (RMS) patients, generally characterized by a very good outcome. Twenty pediatric patients were treated over a 25-year period using the protocols of the Italian and German Soft Tissue Sarcoma Cooperative Groups. Clinical characteristics at presentation were much the same as in non-alveolar patients. The proportion of patients with alveolar histotype (8%) in paratesticular site was lower than in the general RMS population (20-30%). With a median follow-up of 122 months, 5-year EFS and OS were 78 and 89%, respectively. Our data suggest a distinctly better clinical behavior of paratesticular alveolar RMS than when the disease occurs at other sites. These patients were more intensively treated than the embryonal cases, however, so-although a treatment intensity reduction may be desiderable-the idea of eliminating the alkylating agents (as in low-risk embryonal paratesticular RMS) must be considered with great caution.

V03-12 ROBOTIC-ASSISTED LAPAROSCOPIC RPLND FOR PARATESTICULAR RHABDOMYOSARCOMA USING 5 MM INSTRUMENTS IN PEDIATRIC SETTING

A rare case: paratesticular leiomyosarcoma