Recurrent Cervical Sarcoma Botryoides in a 3-Year-Old Female: Approach in a Limited Resource Setting

Abstract

BACKGROUND Sarcoma botryoides, known as embryonal rhabdomyosarcoma (ERMS), is a malignant tumor which arises from embryonic muscle cells. The incidence of ERMS in the uterine cervix rarely occurs at a very young age. With sufficient resources, management of this disease is not difficult. However, in limited resources settings, such as in Indonesia, the situation is more challenging. This case report aims to highlight the difficulties encountered in diagnosing and treating patients with sarcoma botryoides. CASE REPORT A 3-year-old female patient came the outpatient clinic of our hospital with a protruding mass from her vagina resembling a bunch of grapes which easily bled. She underwent surgery to remove the mass. After the procedure, she did not return to the hospital for the recommended adjuvant chemotherapy treatment due to limited funds. Three months later, she came to the outpatient clinic with the same complaint, despite smaller size. Due to limited resources, we only evaluated the metastasis using chest x-ray and did not perform intra-operative biopsy. In the second surgery, a wide excision with 1-2 cm margin was performed, followed by adjuvant chemotherapy for 6 series. We achieved a satisfactory outcome in this case, and 18 months after the surgery, the patient was still in remission. CONCLUSIONS Sarcoma botryoides is a rare malignancy. The effective treatment for sarcoma botryoides is wide excision with safe margin of 1-2 cm, followed by 6-12 cycles of vincristine, actinomycin D, and cyclophosphamide (VAC) regiment as an adjuvant chemotherapy. A family's understanding of the treatment plan is important to achieve desired outcomes. Even with limited resources, this malignancy can still be properly treated.

Figure 1.

Protruding vaginal mass during the first visit.

Figure 2.

Recurrent mass 3 months after the first surgery.

Figure 3.

Anti-desmin antibody showed polypoid-shape tissue covered in squamous epithelial. There was mesenchymal tumor growth with tightly and loosely packed arrangements of alternating zones. Beneath the epithelial layer, there was hypercellular area of rounded-nuclear hyperchromatic spindle-shaped cell, arranged eccentrically with eosinophilic cytoplasm forming the cambium layer. There were also spread of inflammatory cells. (A) 100× magnification. (B) 400× magnification.

Figure 4.

Anti-myogenin antibody gave the positive result in tumor cell nuclei and showed the typical sign of Nicholson cambium layer. (A) 100× magnification. (B) 400× magnification.