We reported the case of 17-year-old male patient with paratesticular rhabdomyosarcoma with progressive paraaortic lymphnodes, treated with debulking paraaortic surgery. Despite being difficult, the surgery was performed by an expert surgeon. Careful dissection also helped save the patent’s kidney. One year after surgery, the patient was last seen with no sign of relapse.
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. It accounts for 5–10% of all malignant tumors. It arises from primordial mesenchymal cells with different levels of skeletal muscle differentiation [7].
Paratesticular localization represents only 7% of all RMS [2]. It may arise from the spermatic cord, epididymis, or testicular tunics [7].
The age distribution is bimodal, with peaks in childhood (1–5 years) and adolescence (16–18 years) [6].
The symptoms of paratesticular RMS are non-specific [2]. It often manifests as a painless scrotal mass but can also present as bruising, hydrocele, or hernia [6].
Scrotal emergencies (testicular torsion, epididymal–orchitis, mumps orchitis) are the primary differential diagnoses and must be excluded [2, 6].
B-HCG and alpha-fetoprotein (AFP) tumor markers are usually not elevated in the case of paratesticular RMS [6].
Testicular ultrasonography is the first line preferred imaging technique [5, 6], and it is required to distinguish between testicular and paratesticular lesions [6]. In 80% of cases, it reveals a mass of heterogenous echostructure with inguinoscrotal extension [1] and increased vascularity on color Doppler [2].
Para testicular RMS can spread through the blood or the lymphatic system. The most common metastatic sites are the lung, liver, and bone. The paraaortic lymph nodes are reportedly involved in 26–43% of cases [5].
A thoracic–abdominal–pelvic CT scan is necessary as part of the extension assessment.
It is essential to get cross-sectional imaging of the retroperitoneum at the start of the process to look for patients with enlarged retroperitoneal lymph nodes (RPLN) [8]. The most common histological subtypes of RMS are alveolar and embryonal. Pathologically, the embryonic variant is characterized by poorly differentiated cells and rhabdomyoblasts with abundant eosinophilic cytoplasm[2].
The rhabdomyoblast is the characteristic cell for diagnosis. When rhabdomyoblasts are absent, immunohistochemical studies with a panel of antibodies containing myosin and desmin are used [9].
Loss of heterozygosity on chromosome 11’s short arm is a cytogenetic characteristic of this malignancy [2].
The management strategy is well codified and depends on the tumor stage and the prognostic group [3].
For tumors in the paratesticular or spermatic cord area, a radical orchidectomy should be done through an inguinal approach. Tumor resection should not be done through the scrotum. En bloc resection should be done for tumors invading scrotal skin. Testicle-preserving approaches should be avoided [8].
All patients, regardless of age, underwent an RPLN assessment if diagnosed with paratesticular rhabdomyosarcoma [10].
Regardless of imaging results, all patients over 10 years of age should have an ipsilateral infrarenal nerve-sparing surgical RPLN assessment. Patients who are under 10 years of age and have not had any radiographic nodal enlargement should not have a surgical RPLN assessment [8]. Occult metastases can be eradicated with multidrug therapy. Therefore, it is recommended for all prognosis groups with a significant improvement in overall survival (OS) and progression-free survival [11]. In the case of residual paraaortic masses after chemotherapy and radiation therapy and the case of negative PET CT, most authors do not recommend surgery due to its high morbidity [10]. However, in the case of positive PET CT, surgery in expert centers should be considered a viable option [12]. Hamilton et al. [12], in their analysis of the SEER database, found a statistically significant improvement in the 5-year overall survival of adolescent patients treated with RPLN (92%) as compared with those who did not undergo RPLN (64% P = 0.003).However, RPLN did not improve 5-year OS among children (98% versus 94%; P = 0.42) or adults (70% versus 53%, P = 0.64). Rhee et al. [13], in the update on pediatric rhabdomyosarcoma from the APSA cancer committee, stated that aggressive surgical resection may be indicated for local or regional recurrence, with complete resection improving overall survival from 8% to 37%. In our case, surgery was decided on the basis of the impossibility of performing an entire course of treatment (chemotherapy and radiation), the positive PET CT, and the growth of the residual mass. The main difficulty in our case was the intimate contact between the paraaortic mass, the left ureter, and the renal vessels. With the presence of a trained surgeon, good knowledge of anatomy, and careful dissection, the complete resection of the mass was performed without harming the left kidney. This case also highlights the importance of treating these patients in an expert center.
In the treatment of rhabdomyosarcoma, several chemotherapy regimens have been employed. The most widely used combinations are ifosfamide, vincristine, etoposide (IVE), ifosfamide, vincristine, dactinomycin (IVA) or vincristine, dactinomycin, cyclophosphamide (VAC) [11]. Radiotherapy is an essential therapeutic tool in treating rhabdomyosarcoma because it improves local control [11].
