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.

: 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

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.

OBJECTIVES: To analyze the clinicopathological features of maxillofacial granular cell tumors (GCT) with the aid of immunohistochemical staining. METHODS: Seven cases of maxillofacial GCT were retrospectively collated, and the microscopic morphology of maxillofacial GCT was analyzed. The expression of S-100, neuron-specific enolase (NSE), SOX-10, CD68, actin, desmin, and Ki-67 in GCT was detected by immunohistochemical staining. The cases were observed in the follow-ups after clinical treatment. RESULTS: All seven GCT tumors lacked envelopes and were poorly defined. Microscopically, the sizes of the tumor cells were large and appeared with inconspicuous cell membranes, forming a syncytium-like appearance. The cytoplasm was filled with characteristic eosinophilic granules. The immunohistochemical results showed that six cases were NSE-positive, five cases were S-100-positive, seven cases were CD68-positive, five cases were SOX-10-positive, one case was actin-positive, and seven cases were desmin-negative. The Ki-67 index did not exceed 5% in all cases. In the follow-up sessions, none of the six cases presented a recurrence. CONCLUSIONS Maxillofacial GCT has a characteristic histological structure. Immunohistochemical S-100, CD68, and other indicators can assist in diagnosis, and the prognosis is good after clinical resection.
Humans ; Ki-67 Antigen/metabolism* ; Granular Cell Tumor/surgery* ; Retrospective Studies ; Actins/metabolism* ; Desmin/metabolism* ; S100 Proteins/metabolism*

Inflammatory myofibroblastic tumor （IMT） is a rare mesenchymal soft tissue tumor characterized by borderline or low-grade malignancy. It is rare childhood tumor with an average age of onset of 10 years old. It is even rarer in infants and toddlers, and the etiology and pathogenesis of this tumor are still unclear. The clinical presentation of IMT is non-specific and are related to the location of the tumor. When the tumor compresses adjacent organs, it can cause pain and functional impairment. According to the current literature, IMT is most commonly found in the digestive and respiratory systems, but also occasionally occur in the genitourinary system, head and neck, and limbs. At present, there have been no reports of nasopharyngeal IMT involving nasal cavity of infants and toddlers at home and abroad.This article reports a case of a massive inflammatory myofibroblastic tumor involving the nasal cavity and nasopharynx in an infant. Plasma-assisted minimally invasive surgery was performed through multiple surgical approaches and achieved satisfactory therapeutic results. This case report may provide valuable reference for the treatment of similar diseases.
Humans ; Infant ; Granuloma, Plasma Cell/pathology* ; Nasopharynx/pathology* ; Neoplasms, Muscle Tissue ; Soft Tissue Neoplasms

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*