Primary intratesticular rhabdomyosarcoma in children: a case report and review of the literature

Abstract

Background: The importance of this paper is to help to emphasize the importance of chemotherapy for children with pure intratesticular rhabdomyosarcoma after radical inguinal orchiectomy is done as first treatment of rhabdomyosarcoma. The information provided in this paper about the follow-up outcomes of the patient described in this paper, it highlights that, recurrence and even metastasis of intratesticular rhabdomyosarcoma in children are more likely to occur if surgery it not combined with chemotherapy.

Case presentation: Herein, we present a 6-year old African male child with a 3 months history of a painless right intratesticular tumour. The tumour was poorly vascularized and was in continuity with the spermatic cord. Pelvic computer tomography (CT) scan showed a heterogeneous mass with well-defined margins without microcalcification and multiple bilateral inguinal enlarged lymph nodes were noticed without pelvic lymphadenopathy. The tumour measured 3.8 × 2.8 × 3.9 cm. The tumour marker panel showed: lactate dehydrogenase of (472 UI/l), alpha-fetoprotein (1.43 UI/ml) and human chorionic gonadotrophin beta (2.9 mUI/ml). Microscopically, the tumour was composed of small to medium size undifferentiated cells. These were oval to spindle, hyperchromatic cells to stromal myxoid degeneration were noted. Tunica albuginea and rete testis both were infiltrated by tumour. The tumour showed high mitotic count which measured 50 mitoses per 10 High Power Field (HPF). The diagnosis of rhabdomyosarcoma (RMS) was confirmed by immunohistochemistry (IHC) testing using myoD antibody which showed strong and diffuse intranuclear staining of the tumour cells. Currently, he is on cyclophosphamide and vincristine chemotherapy regime and his condition has improved much.

Conclusions: The experience obtained from the index case is crucial for the management of patients with intratesticular rhabdomyosarcoma which should always make sure that radical inguinal orchiectomy is covered by chemotherapy and/or radiotherapy. This will potentially lower the possibilities of recurrence and/or metastasis of the tumour, hence improving the prognosis of the patients. We report the clinical, radiological, and laboratory characteristics as well as the outcome of the patient.

Keywords: Case report; Children; Intratesticular; Rhabdomyosarcoma.

Fig. 1

Computer tomography scan images for sagittal (a), coronal (b) and Scout views (c). The arrow is pointing to a well defined homogenous mass replacing the right testis for images a and b while the left testis and the penile shaft were preserved particularly for image c

Fig. 2

Gross appearance of the testicular mass. The tumour is white-tan, lobulated and is growing by pushing into the tunica vaginalis

Fig. 3

a Infiltration of the testicular tissue by the tumour cells and proliferation of vascular structures. The tumour growth is diffuse and the cells are spindle (haematoxylin and eosin stain, ×400). b The tumour cells are spindle and roundish with hyperchromatic (haematoxylin and eosin stain, ×200). c Proliferation of rhabdomyoblast cells. The cells are hyperchromatic, pleomorphic with irregular coarse nuclear chromatin and abundant eosinophilic cytoplasm. The arrow is pointing to numerous brisk mitotic figures were evident (haematoxylin and eosin stain, ×400)

Fig. 4

immunohistochemistry (IHC) staining of the tumour cells by myoD1 antibody. The tumour cell are diffusely and strongly staining showing intranuclear staining (IHC staining, ×200)