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 GemcitabineDocetaxel 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.

: 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 GemcitabineDocetaxel 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.
rhabdomyosarcoma ; adolescent

This is a case of malignant melanoma with rhabdomyosarcomatous differentiation presenting as a conjunctival mass in a 50-year-old male. Melanoma cells were seen to react with desmin, myogenin and vimentin, indicating rhabdomyosarcomatous differentiation. This condition is very rare, with less than twenty cases reported in the literature, which contributes to the limitations in molecular characterization and standard treatment protocols for this entity. This condition has an aggressive course with a poor prognosis.

Objective:To explore the clinical manifestations,the type of pathology, treatment and prognosis of laryngeal rhabdomyosarcoma, and to enhance the understanding of the clinical characteristics of the disease, while improving the diagnosis rateand reducing the misdiagnosis rate, in order to explore effective diagnosis and treatment methods. Methods:A retrospective analysis was conducted on the clinical data of 5 cases of laryngeal rhabdomyosarcoma treated in the First Affiliated Hospital of Zhengzhou University from May 2015 to May 2021. Results:All 5 cases of laryngeal rhabdomyosarcoma were misdiagnosed in the early stage. with tumors mostly occurring in the vocal cords and appearing as smooth mass. The clinical symptoms were mostly hoarseness. According to pathological classification, three cases were embryonic type, one case was polymorphic type, and one case was spindle type.Three patients died due to tumor recurrence, one patient had multiple systemic metastases, and another patient who underwent surgical resection in the early stage and supplemented with postoperative radiotherapy and chemotherapyhas been followed up to date without recurrence. Conclusion:Laryngeal rhabdomyosarcoma has low incidence rate, high malignancy degree and poor prognosis. It is easy to be misdiagnosed as a benign mass. Extensive surgical resection combined with radiotherapy and chemotherapy should be performed as soon as possible after diagnosis.
Adult ; Humans ; Retrospective Studies ; Neoplasm Recurrence, Local/therapy* ; Larynx/pathology* ; Rhabdomyosarcoma/therapy* ; Vocal Cords/pathology*

Humans ; Neoplasms ; Rhabdomyosarcoma

Carcinoma, Acinar Cell ; Humans ; Rhabdomyosarcoma/surgery* ; Stomach ; Stomach Neoplasms/surgery*

Objective: To investigate the clinical characteristics, treatment and prognosis of malignant rhabdoid tumor (MRT) in children. Methods: Clinical data total of 18 children with MRT treated in the Department of Hematology and Oncology, Children's Hospital, the First Affiliated Hospital of Zhengzhou University between June 2015 to June 2021 were analyzed retrospectively. The patients were grouped according to age, gender, tumor type, clinical stage and other factors.Progression free survival (PFS) and overall survival (OS) were calculated by Kaplan-Meier method, survival differences among different groups were compared by Log-rank test, and prognostic factors were analyzed by Cox regression model. Results: Among the 18 patients, there were 5 males and 13 females. The age of disease onset was 30.5 (12.0, 75.0) months, the tumor diameter was (80±29) mm, and no integrase interactor 1 (INI-1) expression was detected by immunohistochemistry. There were 7 cases of malignant rhabdoid tumor of the kidney (MRTK), 6 cases of atypical teratoid rhabdoid tumor (ATRT) and 5 cases of extrarenal extracranial rhabdoid tumor (EERT). At the time of early diagnosis, 12 patients were clinically stage Ⅲ-Ⅳ, 11 patients had local or distant metastasis, and 4 patients had metastasis during treatment. Surgical excision is the preferred treatment. There were 3 cases with preoperative puncture biopsy, 13 cases with complete resection, 4 cases with partial resection, and 1 case without operation. Thirteen patients were treated with the domestic conventional chemotherapy regimen for Wilms' tumor, medulloblastoma and rhabdomyosarcoma, and 5 patients were treated with the international conventional chemotherapy regimen. Nine patients received radiotherapy, including 1 case of MRTK, 4 cases of ATRT and 4 cases of EERT. By the end of follow-up in January 2022, 7 patients survived and 11 patients died. The 3-year PFS and OS rates were (8±8) % and (14±12) %. Log-rank test showed that the 5-year OS of EERT group was higher than ATRT and MRTK groups (χ²=16.31, P<0.001), the tumor diameter <80 mm group was higher than that of the ≥80 mm group (χ²=4.49, P=0.034), and the radiotherapy group was higher than no radiotherapy group (χ²=3.97, P=0.046). The differences were statistically significant. There was no significant difference in the influence of tumor type, age, tumor diameter, radiotherapy and chemotherapy on OS by Cox regression model (all P>0.05). Log-rank test showed that the 3-year PFS of EERT group was higher than ATRT and MRTK groups (χ²=11.14, P=0.004),>3 years group was higher than ≤3 years group (χ²=10.10, P=0.001), the differences were statistically significant. Tumor type, clinical stage, tumor diameter, age, tumor rupture and radiotherapy were included in the Cox regression model, and the results showed that clinical stage (HR=0.49, 95%CI 0.26-0.94, P=0.031), tumor diameter (HR=8.67, 95%CI 1.84-40.89, P=0.006), age (HR=0.01, 95%CI 0.00-0.15, P=0.001) had statistical significance on PFS. Conclusions: MRT is one of the most aggressive and fatal cancers in early childhood and infancy. There is no standard treatment and the prognosis is extremely poor. Clinical stage, tumor size and age are risk factors for disease progression.
Child ; Child, Preschool ; Female ; Humans ; Kidney Neoplasms ; Male ; Prognosis ; Retrospective Studies ; Rhabdoid Tumor/therapy* ; Rhabdomyosarcoma, Embryonal ; Survival Rate

Testicular rhabdomyosarcoma is relatively rare in testicular tumors, but the age of patient is relatively young and the degree of malignancy is high. Therefore, this article introduces 4 cases of testicular rhabdomyosarcoma who were admitted to Peking University Third Hospital from May 1994 to February 2019, and reviews the literature to improve the diagnosis and treatment of this disease. The average age of the 4 patients was 17.5 years (14-21 years), the average hospital stay was 22.0 d (17-31 d), and the average body mass index was 19.6 kg/m² (14.7-25.8 kg/m²). All the patients underwent routine preoperative blood and urine routine, biochemical tests, as well as serum tumor markers. Preoperative examinations also included chest radiograph, electrocardiogram, ultrasound of the scrotum and groin, and abdominal enhanced CT. Lung CT or other examinations were performed if necessary. The median serum human chorionic gonadotropin (HCG) of the 4 patients was 0.20 IU/L (0.06-0.86 IU/L) (all normal), and the median serum alpha-fetoprotein (AFP) was 1.03 g/L (0.65-1.66 g/L) (all normal). The average maximum diameter of the tumor was 10.0 cm (4.5-15.0 cm). Testicular rhabdomyosarcoma was mainly diagnosed by pathology. The main treatment was radical orchiectomy combined with retroperitoneal lymph node dissection, with or without postoperative adjuvant chemotherapy. The clinical manifestations of the patients with testicular rhabdomyosarcoma had no specific characteristics, but most patients were young at onset with mainly painless masses in the testicles, which were already large when they were found. Patients with testicular rhabdomyosarcoma have a poor prognosis, most of whom recur within two years. Because of the small number of cases of testicular rhabdomyosarcoma, there is no standard treatment currently. It is recommended that patients with testicular rhabdomyosarcoma undergo radical testicular resection combined with retroperitoneal lymph node dissection. Retroperitoneal lymph node metastasis is an important prognostic factor, and patients with postoperative adjuvant chemotherapy can still survive for a longer time. If local recurrence or limited metastasis is found after operation, local resection and salvage radiotherapy are feasible.
Adolescent ; Biomarkers, Tumor ; Humans ; Lymph Node Excision ; Male ; Rhabdomyosarcoma/therapy* ; Scrotum ; Testicular Neoplasms

Humans ; Perineum ; Rhabdomyosarcoma/diagnosis*

Animals ; Brachytherapy ; Combined Modality Therapy ; Humans ; Male ; Prognosis ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar/drug therapy*