Arenos Carl Lawrence C, Fernando Gracieux Y, Dy Pauline Mae R, Lim Maria Cecilia F, Causapin Joseph D
Division of Medical Oncology, Department of Medicine, Philippine General Hospital, University of the Philippines Manila.
Department of Laboratories, Philippine General Hospital, University of the Philippines Manila.
Acta Med Philipp. 2025 Jun 30;59(8):94-101. doi: 10.47895/amp.vi0.9280. eCollection 2025.
Vulvar Rhabdomyosarcoma (VR) is a rare gynecological cancer primarily found in children. This case report discusses the diagnosis, treatment, and management complexities of a 19-year-old patient with a slow-growing vulvar lesion. A 19-year-old female with obesity and non-alcoholic fatty liver disease presented with a left vulvar lesion measuring 11 x 7 x 7 cm that was noticed five months ago. Core needle biopsy of the lesion revealed findings consistent with rhabdomyosarcoma. A Positron Emission Tomography-Computed Tomography (PET-CT) scan showed a hypermetabolic 8.3 x 6.7 x 6.7 cm mass in the left vulvar area, extending to the vagina, rectal wall, and anal region along with enlarged left inguinal lymph nodes. The patient was treated with the Intergroup Rhabdomyosarcoma Study-IV (IRS-IV) protocol for 16 weeks with vincristine, dactinomycin, and cyclophosphamide. Concurrent chemoradiotherapy was administered between weeks 9-14, followed by continuation chemotherapy until week 28. Interim PET-CT scan prior to concurrent chemoradiotherapy revealed a reduced mass size to 3.8 x 2.8 cm and resolved left inguinal lymphadenopathy. Despite completing treatment, the patient reported persistent back pain and mobility issues three weeks later. A subsequent PET-CT scan showed hypermetabolic lesions at vertebral locations C6, T9, T12, and L1-L3, along with the left ischium and bilateral femoral shafts. Thoracic vertebrae biopsy confirmed rhabdomyoblasts. Patient underwent palliative radiotherapy and spinal stabilization then proceeded with second line therapy with 1 cycle of Gemcitabine-Docetaxel but showed progression of symptoms described as persistent bleeding (hematuria) and neutropenia. Further diagnostics done to the patient showed possible bone marrow involvement. Unfortunately, the patient expired owing to symptoms of cancer progression. The management of adult-onset VR presents a significant therapeutic challenge, largely attributable to the scarcity of clinical trials and tailored treatment regimens for this specific age group. Outcomes documented in existing literature for adult VR cases present with recurrence, disease progression, and mortality. The treatment landscape in adults is complicated by comorbidities which may influence both the therapeutic choices and outcomes. Given these intricate challenges, this case echoes the need for research efforts aimed at developing management protocols specifically designed for adults with VR.
外阴横纹肌肉瘤（VR）是一种罕见的妇科癌症，主要发生于儿童。本病例报告讨论了一名19岁、患有生长缓慢的外阴病变患者的诊断、治疗及管理复杂性。一名患有肥胖症和非酒精性脂肪性肝病的19岁女性，出现一个5个月前被发现的左侧外阴肿物，大小为11×7×7厘米。对该肿物进行的粗针活检结果显示与横纹肌肉瘤相符。正电子发射断层扫描-计算机断层扫描（PET-CT）显示左侧外阴区域有一个8.3×6.7×6.7厘米的高代谢肿物，延伸至阴道、直肠壁和肛门区域，同时左侧腹股沟淋巴结肿大。该患者按照横纹肌肉瘤协作组研究-IV（IRS-IV）方案接受了16周的长春新碱、放线菌素D和环磷酰胺治疗。在第9至14周期间进行了同步放化疗，随后继续化疗直至第28周。同步放化疗前的中期PET-CT扫描显示肿物大小缩小至3.8×2.8厘米，左侧腹股沟淋巴结病消退。尽管完成了治疗，但患者在3周后报告持续存在背痛和活动问题。随后的PET-CT扫描显示在C6、T9、T12和L1-L3椎体部位以及左侧坐骨和双侧股骨干有高代谢病变。胸椎活检证实存在横纹肌母细胞。患者接受了姑息性放疗和脊柱固定，然后进行了吉西他滨-多西他赛1个周期的二线治疗，但出现了如持续出血（血尿）和中性粒细胞减少等症状进展。对患者进行的进一步诊断显示可能存在骨髓受累。不幸的是，患者因癌症进展症状而死亡。成人期VR的管理面临重大治疗挑战，这在很大程度上归因于针对该特定年龄组的临床试验和定制治疗方案的稀缺。现有文献中记录的成人VR病例的结果包括复发、疾病进展和死亡。成人的治疗情况因合并症而变得复杂，合并症可能会影响治疗选择和结果。鉴于这些错综复杂的挑战，本病例凸显了开展旨在制定专门针对成人VR的管理方案的研究工作的必要性。
