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.

OBJECTIVE: To explore the feasibility and effectiveness of repairing surgical defect in pharyngo-laryngeal malignant tumor with free rectus femoris flap. METHODS: The clinical data of 34 patients with surgical defects in pharyngo-laryngeal malignant tumor who met the selection criteria between July 2014 and August 2024 were retrospectively analyzed. There were 25 males and 9 females, aged 25-82 years, with a median age of 54 years. The disease duration ranged from 2 months to 2 years, with a median of 7 months. The tumor locations included the oropharynx, hypopharynx, cervical esophagus, and larynx. Pathological types included squamous cell carcinoma (29 cases), myoepithelial carcinoma (2 cases), adenoid cystic carcinoma (1 case), and diffuse large B-cell lymphoma (2 cases). TNM staging: 16 cases of T ₄N ₁M ₀, 3 cases of T ₄N ₂M ₀, 3 cases of T ₄N ₀M ₀, 10 cases of T ₃N ₁M ₀, and 2 cases of T ₃N ₀M ₀. The 2017 American Joint Committee on Cancer (AJCC) staging was stage Ⅲ in 2 cases and stage Ⅳ in 32 cases. The blood supply of the proximal rectus femoris muscle was observed by enhanced CT of the lower limb vessels before operation, and the surgical defects ranged from 3.0 cm×2.0 cm to 12.0 cm×8.5 cm. The blood supply and perforators of rectus femoris muscle were explored during operation, and the free rectus femoris flap pedicled with the direct vascular stem of rectus femoris muscle was used to repair the defect. For the patients with pharyngeal fistula or obvious neck swelling after operation, the blood supply of the flap was analyzed by vascular enhanced CT to determine the corresponding strategies of nutritional support, anti-infection, dressing change and drainage. Radiotherapy and chemotherapy were supplemented in 27 patients with lymph node metastasis after operation. RESULTS: All the 34 patients were followed up 1-10 years, with an average of 3 years. The flap was found to be necrotic by fibrolaryngoscopy at 1 week after operation in 2 cases, and the incision healed after dressing change and nutritional support, and no reoperation was performed. The flap was in good condition at 1 week after operation in 4 cases, and the signs of gradual necrosis of the flap were found within 1 month after operation, of which 2 cases were healed after dressing change, 1 case was removed the necrotic tissue by reoperation, and 1 case was healed after pectoralis major myocutaneous flap was used to repair the pharyngeal tissue defect. The flaps survived in 28 cases, including 4 cases of pharyngeal fistula, which healed by dressing change. Twenty-two cases achieved satisfactory results in swallowing or phonation. Two patients with total laryngectomy and voice reconstruction underwent reoperation to seal the voice tube because of postoperative aspiration. During the follow-up, 1 case had tracheal stomal recurrence, 2 cases had bone metastasis, and 1 case had bone and lung metastasis. CONCLUSION The free rectus femoris flap has good flexibility, the volume of the flap is easy to adjust, and the incision of the donor site is concealed, which is expected to become a new choice for the repair of the surgical defect in pharyngo-laryngeal malignant tumor.
Humans ; Male ; Middle Aged ; Female ; Aged ; Adult ; Plastic Surgery Procedures/methods* ; Retrospective Studies ; Laryngeal Neoplasms/pathology* ; Aged, 80 and over ; Pharyngeal Neoplasms/pathology* ; Free Tissue Flaps/blood supply* ; Quadriceps Muscle/transplantation* ; Surgical Wound/surgery* ; Treatment Outcome

OBJECTIVES: Hysterectomy remains the only definitively effective treatment for diffuse uterine leiomyomatosis (DUL). However, no standardized management strategy exists for DUL patients wishing to preserve fertility. This study summarizes and analyzes 5 cases of individualized treatment in DUL patients desiring fertility preservation, aiming to provide a clinical reference for personalized management of similar patients. METHODS: We retrospectively analyzed the clinical data of 5 DUL patients with fertility intentions admitted to the Department of Obstetrics and Gynecology at Third Xiangya Hospital of Central South University. To preserve fertility, individualized treatment plans were selected based on clinical manifestations and fibroid distribution. One patient received high-intensity focused ultrasound (HIFU); one underwent hysteroscopic myomectomy (HM) combined with laparoscopic myomectomy (LRM); one underwent HIFU combined with HM and LRM; one received drug therapy combined with staged HM; and one underwent HIFU combined with staged HM and drug therapy. Treatment outcomes and pregnancy results were analyzed. RESULTS: After treatment, all 5 patients showed marked improvement in menstrual volume or dysmenorrhea symptoms and significant reduction in uterine volume; mild intrauterine adhesions occurred in 3 cases. All 5 patients achieved successful pregnancy. One patient with chronic hypertension developed severe preeclampsia at 34 weeks and underwent cesarean section, while the remaining 4 delivered at term by cesarean section. Three cases of placenta accreta and 2 cases of postpartum hemorrhage occurred. During long-term follow-up, one patient underwent hysterectomy 2 years postpartum due to increased menstrual volume, while the other 4 remained stable. CONCLUSIONS Individualized treatment tailored to DUL patients' conditions can preserve fertility, support successful pregnancy, and achieve favorable pregnancy outcomes.
Humans ; Female ; Pregnancy ; Leiomyomatosis/therapy* ; Uterine Neoplasms/therapy* ; Adult ; Retrospective Studies ; Fertility Preservation/methods* ; Hysterectomy ; Uterine Myomectomy/methods* ; High-Intensity Focused Ultrasound Ablation ; Pregnancy Outcome

Embryonal rhabdomyosarcoma of the uterus is a rare condition with only a few cases documented. Exceedingly rare, however, is its concomitant incidence with uterine inversion. The infrequency with which genital tract sarcoma with uterine inversion is encountered makes the diagnosis and management a formidable challenge. The present case reports a 12-year-old nulligravida who complained of a rapidly growing introital mass of 3-month duration. Suspicion of nonpuerperal uterine inversion was confirmed by imaging, and malignancy was proven through adequate tissue sampling. While there is no unified protocol in the management of prolapsed genital tract sarcomas, the complete inversion of the corpus necessitated surgery. In the case presented, exploratory laparotomy and total hysterectomy through a double setup, abdomino-vaginal approach was done. The case illustrates the diagnostic, therapeutic, and ethical dilemmas in handling an aggressive tumor in an adolescent. Early recognition and a multidisciplinary approach are extremely crucial in ensuring improved prognosis and holistic treatment.

BACKGROUND

Hysteroscopic myomectomy is regarded as the gold standard in the management of intracavitary myomas. With its increased use, it is essential to explore its effectiveness for better therapeutic planning and patient selection.

This study aimed to determine the effectiveness of hysteroscopic myomectomy performed in a tertiary hospital in the Philippines.

A retrospective cross-sectional study of women who underwent hysteroscopic myomectomy in 5 years was performed.

A total of 167 women were included. Completeness of resection was achieved in 88.46% of the cases. In the final multivariate model, older age, pretreatment with gonadotropin-releasing hormone (GnRH) agonist, and lesser total fluid input were associated with increased odds of complete resection. Submucous myoma at least 3 cm in size had greater total fluid input, greater blood loss, the presence of complications, and a greater need for transfusion. Pretreatment with GnRH agonists had more International Federation of Gynecology and Obstetrics Grade 1 and 2 myoma, higher frequency of ≥3 myomas, lower frequency of complete resection, and increased operative time compared to those without pretreatment with GnRH agonists. Pretreatment with other hormonal therapy had a lesser need for transfusion compared to those without pretreatment with hormonal therapy.

Hysteroscopic myomectomy is a generally safe and effective procedure for the treatment of submucous myomas. The odds of complete resection are greater with older age and decreased with pretreatment with GnRH agonist and more distention fluid used. Larger submucous myoma was associated with greater total fluid input and blood loss, more complications, and greater need for transfusion. Pretreatment with GnRH agonist had no significant benefit and was associated with longer surgical time and lesser complete resection.

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.

: 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

Humans ; Female ; Fumarate Hydratase/genetics* ; Kidney Neoplasms ; Carcinoma, Renal Cell ; Leiomyomatosis ; Uterine Neoplasms

Humans ; Sarcoma, Alveolar Soft Part/secondary* ; Lung Neoplasms/pathology* ; Soft Tissue Neoplasms/pathology*

Objective: To investigate the clinicopathologic and molecular characteristics of fumarate hydratase (FH) deficient uterine leiomyoma. Methods: Eighty cases of FH deficient uterine leiomyoma were diagnosed from April 2018 to September 2022 in Department of Pathology, Peking University Third Hospital. Sanger sequencing of FH gene exons (exon 1-10) were performed on tumor tissues and matched non-tumor tissues/peripheral blood for all cases. FH immunohistochemistry were performed in 74 cases; S-(2-succino)-cysteine (2SC) were also detected by immunohistochemistry in five cases. Results: Patients' age ranged from 18 to 54 (36.0±7.5) years, with more than 60% exhibiting clinical symptoms of multiple and large leiomyomas (the median diameter was 70 mm). More than four histologic features, including staghorn vasculature, alveolar-pattern edema, bizarre nuclei, oval nuclei arranged in chains, prominent eosinophilic nucleoli with perinucleolar haloes and eosinophilic intracytoplasmic globules were observed in 98.5% (67/68) patients. The immunohistochemical sensitivity of FH and 2SC were 97.3% and 100%, respectively. Based on the Sanger sequencing results, the cases were divided into germline variant group (31 cases), somatic variant group (29 cases) and no variant group (20 cases). Sixty-nine percent (20/29) of the patients with FH germline variation had clear family history. Conclusions: Clinical features, histological morphology, FH and 2SC immunohistochemistry and Sanger sequencing have their own significance and limitations in differential diagnosis of FH deficient uterine leiomyoma. In clinical practice, the above information should be fully integrated and studied for accurate pathologic diagnosis and selection of patients with FH germline variation.
Female ; Humans ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Fumarate Hydratase/genetics* ; Uterine Neoplasms/pathology* ; Leiomyoma/pathology* ; Germ-Line Mutation ; Diagnosis, Differential ; Leiomyomatosis/pathology* ; Carcinoma, Renal Cell/diagnosis*