Emergency Radiation Therapy for Hemorrhage in Botryoides Sarcoma: A Pediatric Case Report

Introduction

Botryoid rhabdomyosarcoma is derived from the Greek term “botryoid”, which means “a cluster of grapes-like”, describing its clinical manifestation and radiological findings. It is also known as embryonal rhabdomyosarcoma, a specific type of rhabdomyosarcoma, which is a cancerous tumor that develops from embryonic muscle cells [1,2]. This type of cancer, known as soft-tissue sarcoma, most frequently occurrs in children and young adults. It makes up approximately 4% to 6% of all cancers in this age range [1,2], specifically children aged 0–14 years, with 3% of cancer cases and 1% of cases among adolescents and young adults aged 15–19 years. The 5-year survival rate is around 70% for children and up to 50% for adolescents and young adults [3]. There are a few rhabdomyosarcomas that are primarily located in the cervix of girls or children, with the vagina being the most common primary site [4–6]. Typically, it is documented as a tumor located in the vagina of newborns and rarely in the cervix of the uterus in the female reproductive system [7]. We report a case of botryoid rhabdomyosarcoma occurred in a girl, presenting with a mass protruding out of the vagina. While it typically presents with symptoms such as vaginal bleeding or urinary obstruction, hemorrhage is a rare but potentially life-threatening complication. MRI is the primary imaging modality in rhabdomyosarcoma due to its superior ability to assess soft-tissue changes [8]. The management of hemorrhage in botryoid rhabdomyosarcoma can be challenging, often necessitating a multidisciplinary approach. This case report highlights the use of emergency external radiation therapy to successfully manage recurrent bleeding in a patient with botryoid rhabdomyosarcoma.

Case Report

A 15-year-old girl was admitted to our hospital with a main concern of vaginal bleeding and protruding mass in her vagina for 2 months (Figure 1A). The mass was initially small, and she was brought to the hospital only after realizing its rapid growth. There was no significant medical history or family history of cancer. On gynecologic examination, a polypoid mass measuring 8×7 cm was visualized protruding from the vaginal introitus, with a tendency to bleed. Magnetic resonance imaging (MRI) confirmed a massive malignant mass originating from the vaginal introitus, infiltrating the cervix (Figure 1B), extending into the uterus, and obliterating the uterus, suggestive of a malignant tumor. There was no infiltration of the mass to the bladder or rectum, and no pelvic lymph node enlargement was observed. There was left hydronephrosis due to mass compression. Histopathology examination confirmed the diagnosis of botryoid rhabdomyosarcoma (Figure 2).

Given the suspicion of malignancy and recurrent vaginal bleeding, the patient underwent excessive repeated transfusions while waiting results of a histopathology examination and MRI. As the disease progressed, the presence of the mass led to left-sided hydronephrosis and acute kidney injury. Following diagnosis, she underwent emergency external radiation therapy to achieve hemostasis, which contributes to local tumor control by inducing tumor regression and reducing the risk of recurrent bleeding (Figure 3A, 3B). After a single radiation therapy session with a dose of 2 gy over 30 minutes, the bleeding was significantly reduced. The cytotoxic effects of radiation can lead to tumor shrinkage and fibrosis, thereby stabilizing or improving the patient’s clinical condition in the short term [9]. After the emergency condition had been solved, the patient was planned for chemotherapy with a vincristine, actinomycin, and cyclophosphamide (VAC) regimen. She is currently undergoing the third cycle of chemotherapy, with a good response, and is planned to undergo complete tumor resection to improve survival.

We report the case of a 15-year-old girl with symptoms of vaginal bleeding and protruding mass from the vagina for 2 months, with no history of cancer. Based on the histopathology and MRI results, she was diagnosed with botryoid rhabdomyosarcoma. To manage bleeding, she received emergency external radiation therapy and is undergoing the third cycle of chemotherapy using the vincristine, actinomycin, and cyclophosphamide (VAC) regimen.

Discussion

This case report emphasizes the use of emergency external radiation therapy to reduce a patient’s morbidity, particularly excessive bleeding and complications that can occur with surgery. Some reports have discussed combination therapy, such as surgery, chemotherapy, and radiotherapy. However, there are few reports on use of emergency external radiation therapy in such patients.

Our patient was an adolescent with no family history of cancer. This is consistent with the literature on botryoid rhabdomyosarcoma, which most often affects young people, particularly in early childhood and young adulthood. Vaginal bleeding and a protruding mass from vaginal introitus are the most common clinical manifestations presented in many cases and it is reported in our case as well [10–12].

Histopathological examination is the criterion standard for diagnosing botryoid rhabdomyosarcoma. Recent studies indicated that MRI is useful in assessing the local extent of pelvic rhabdomyosarcoma, as it can delineate tumor extension better than other modalities such as computed tomography (CT) or ultrasonography (USG) [8,13]. In our case, MRI confirmed a large malignant mass originating from the vaginal introitus, infiltrating the cervix, extending into the uterus, and obliterating the uterus, which was suggestive of a malignant tumor. This was confirmed by histopathology, which identified botryoid rhabdomyosarcoma. A study by Chakrabarty et al reported a similar case, in which botryoid rhabdomyosarcoma was diagnosed by histopathology, with CT and USG used as additional modalities [12].

However, our patient received multiple blood transfusions due to bleeding caused by the mass. Emergency external radiation therapy was performed to help achieve hemostasis. Bleeding is one of the emergency conditions in oncologic patients with metastatic malignant tumor (cancer) or advanced-stage local cancer. There are few studies in recent publications in the journals of the European Society for Radiotherapy and Oncology (ESTRO) and the American Society for Therapeutic Radiation Oncology (ASTRO) that specifically explain the management of symptoms and palliative care for cancer patients. One study found that radiation therapy is an effective treatment for controlling bleeding caused by cancers in emergency situations, with approximately 90% of the cases showing successful control of bleeding [14,15].

Currently, multidisciplinary management involving surgery, chemotherapy, and radiotherapy is the cornerstone of treatment for botryoid rhabdomyosarcoma. Surgical resection aims to achieve complete tumor excision while preserving organ function and minimizing morbidity [10,13]. Even with adjuvant chemotherapy regimens, there is no clear consensus on the management of these tumors. Most of the regimens use a combination of 2 or 3 chemotherapeutic agents, such as the VAC regimen, which was also used in our case. The most commonly used chemotherapy regimen given to children and young adults is the combination of vincristine, actinomycin D, and cyclophosphamide (VAC) [10]. A study by Imawan et al also reported the use of vincristine, actinomycin, and cyclophosphamide (VAC regimen) in a patient with botryoid rhabdomyosarcoma [1]. The regimen was administered preoperatively to reduce tumor size and facilitate surgical resection, as well as postoperatively to eradicate residual disease and prevent metastasis [10]. However, in the same study, hemorrhage in botryoid rhabdomyosarcoma posed significant challenges in management, often requiring prompt intervention to prevent morbidity and mortality. Surgical excision is the preferred treatment modality for localized disease, it may not always be feasible, particularly in cases of extensive or inaccessible tumors [10,13]. Radiotherapy is used to treat most high- and intermediate-risk patients [13]. Risk stratification is based on patient age, tumor site and size, PAX-FOXO1 fusion status, the nodal involvement or metastatic disease, and the Intergroup Rhabdomyosarcoma Studies (IRS) Post-Surgical Group staging [10,16,17]. A combination of chemotherapy and radiation therapy without surgery has been reported to be curative of localized vaginal lesions [18]. In such scenarios, radiation therapy can be a valuable adjunct, offering rapid control of bleeding while minimizing the risks associated with surgery.

Radiation therapy is primarily indicated for controlling localized disease, which significantly impacts survival and is an important part of the treatment for pediatric and adolescent rhabdomyosarcoma. Radiotherapy has various types, such as external beam radiation, intensity-modulated radiation, proton therapy, and brachytherapy [13,17]. In the most recent publications collected in one study, the frequency of surgery has decreased and radiotherapy was infrequently used. Currently, with multidisciplinary treatment, there is improvement in the prognosis of botryoid rhabdomyosarcoma, despite its rarity and malignancy, as evidenced by the 95% survival rate at 5 years [10].

External radiation therapy delivers focused high-energy radiation to the tumor site, exerting cytotoxic effects on tumor cells and inducing vascular thrombosis, thereby halting hemorrhage. The rapid onset of action makes it an ideal therapeutic option for managing acute bleeding episodes, providing symptomatic relief and stabilizing the patient’s condition while definitive treatment plans are formulated [9,19].

In our case, the decision to initiate emergency external radiation therapy was based on the patient’s clinical presentation, including the severity of hemorrhage and the inability to achieve hemostasis through conventional measures. While radiation therapy carries its own set of risks, including potential toxicity to surrounding normal tissues such the bladder, rectum, and ovaries, the benefits of rapid hemorrhage control outweighed the risks in this emergency situation.

The integration of tumor resection, chemotherapy, and, in instances of treatment-resistant tumors or residual disease, radiation therapy is a suitable strategy for carefully chosen patients with botryoid rhabdomyosarcoma. This method yields outstanding oncological results with low rates of complications, considering the tumor’s location, stage, and overall characteristics of the patient. However, given that much of the current information is derived from case reports, it is essential to carry out larger studies with extended follow-ups to establish the most effective treatment protocols [10].

Conclusions

Hemorrhage in botryoid rhabdomyosarcoma is a rare but critical complication, requiring prompt intervention to prevent adverse outcomes. While surgical excision remains the cornerstone of treatment, emergency external radiation therapy plays a crucial role in management of acute bleeding, offering rapid symptomatic relief and stabilization of the patient’s condition. Further research is warranted to delineate optimal treatment strategies and refine the role of radiation therapy in this challenging clinical scenario.