Successful management strategy for giant primary hepatic rhabdomyosarcoma in 15-year-old male: a case report.

Primary hepatic rhabdomyosarcoma is an exceedingly rare type of sarcomas. A 15-year-old boy was admitted to the hospital with abdominal pain. Serial investigation showed a giant heterogeneous mass, with a diameter of 15 cm, located in the right lobe of the liver (segment 4a, 4b, 5, 6, 7, 8), with clear margins, non-homogeneous density in computerized tomography and the Positron Emission Tomography Scan Multi Slice revealed a peripherally slightly metabolically active hepatic mass (205 × 134 × 208 mm). Histopathological examination and the Immunohistochemical showed embryonal rhabdomyosarcoma. The management strategy involved multiple cycles of ifosfamide and doxorubicin and monitoring the tumor size, until the tumor size was suitable for the surgery. The management of hepatic rhabdomyosarcoma is a surgical challenge due to the late onset of symptoms, a limited number of reported cases, and poor prognosis. Surgical excision is the treatment of choice for primary hepatic rhabdomyosarcoma and combination between chemotherapy, surgery showed good results.

Scrotal arteriovenous malformation (AVM) successfully resected without angioembolization: A case report.

INTRODUCTION AND IMPORTANCE: The main types of scrotal vascular lesions are varicocele, hemangioma, lymphangioma and arteriovenous malformation (AVM). AVM consists of network between arteries and veins without capillaries. It is the rarest type especially when in scrotum. CASE PRESENTATION: A 24-year-old male patient presented with a skin deformity and painless swelling in the left scrotum. Physical examination revealed this swelling that extended to the inguinal region. Duplex Ultrasound (DUS) followed by Multi-slice Computed Tomography (MSCT) were performed to establish the diagnosis. Management depended on surgical excision without angioembolization. Preoperative sperm analysis showed oligoasthenozoospermia that improves significantly after treatment and 1 year of follow-up. CLINICAL DISCUSSION: Surgical resection of scrotal AVM without embolization has been used in very few cases and has resulted in a satisfactory outcome with no signs of recurrence throughout the follow-up period. CONCLUSION: Based on our experience, surgical excision without embolization is a reasonable alternative approach to treat scrotal AVM in low-income countries alongside avoiding the negative consequences of radiation therapy. Treatment should be considered when fertility is affected.

Rare giant renal artery aneurysm in neurofibromatosis type 1 patient: a case report.

Introduction and importance: Neurofibromatosis type 1 (NF1) is a genetic disorder characterised by multiple neurofibromas, café-au-lait spots, and iris hamartomas. The variety of vasculopathies that can occur in NF1 make it difficult for clinicians to accurately follow-up patients. Most cases of vasculopathies are stenotic, and, in few cases, aneurysms may form. Case presentation: A 35-year-old male presented with extreme left flank pain for the past 2 days. His physical examination revealed whole-body several café-au-lait skin macules, a subcutaneous lesion, and a palpable abdominal mass in the left flank. His laboratory workup was within normal ranges. A multi-slice computed tomography and computed tomography angiogram with contrast outlined a giant left renal artery aneurysm (RAA). A kidney salvage surgery was planned. However, due to ectatic dilatation and large extension of the aneurysm, the affected renal artery branches and renal vein were found unfit for auto-transplantation during the surgical procedure and a total nephrectomy was necessary. Symptoms improved significantly postoperatively and no complications developed. Clinical discussion: RAA is an uncommon finding in NF1 patients. Diagnosis is often dependent on computed tomography angiogram. Management techniques are conservative, endovascular, or surgical. In few surgical cases, a total nephrectomy may be necessary if auto-transplantation is not feasible. Conclusion: Despite its rarity, the diagnosis of RAA should be considered in patients with NF1 presenting with flank pain. Moreover, early screening for renal vasculopathies can evade critical surgical outcomes including a total nephrectomy. Hence, the authors recommend a total vascular workup for these patients, consisting of doppler ultrasound and, if necessary, a multi-slice computed tomography with contrast.

A rare co-occurrence of renal cell carcinoma, bilateral adrenal metastasis and inferior vena cava thrombus: A case report.

INTRODUCTION: The incidence of ipsilateral adrenal metastasis from RCC varies between 1.1 and 10 %, on the other hand, the presence of bilateral adrenal metastasis from solitary RCC is extremely rare, with less than 20 reported cases in the literature. CASE PRESENTATION: A 68-year-old man presented to the clinic with hematuria. Further investigations, contrast CT, showed mass at the right kidney and adrenal gland, a mass on the left adrenal gland and inferior cava thrombosis measuring 3*6 cm. The patient underwent, first, right nephrectomy and adrenalectomy with thrombectomy and IVC plasty. Microscopic examination showed clear cell renal cell carcinoma, while adrenal tissue showed metastatic renal cell carcinoma. The patient was followed up from the oncological, endocrinological and cardiac point of view. After 6 months he underwent left adrenalectomy. Subsequent follow-up showed body free of metastases. CLINICAL DISCUSSION: The co-occurrence of renal cell carcinoma, bilateral adrenal metastasis, and inferior vena cava thrombus is a very rare phenomenon in the literature. Most patients with adrenal metastasis are asymptomatic, and the adrenal function is typically preserved even when malignancy affects bilateral adrenal glands. In addition, patients with adrenal ipsilateral metastases typically have primary renal tumors with poor prognosis. CONCLUSION: The Diagnosis and management are a surgical challenge. Contrast-enhanced CT scan is the preferred imaging modality for renal cell carcinoma. The surgical intervention is mandatory if it can increase survival rate.

A remarkably rare case of Adventitial Cystic Disease of the Popliteal Artery in a 51-year-old Middle Eastern female - A Case Report.

INTRODUCTION AND IMPORTANCE: Adventitial Cystic Disease (ACD) is a vastly rare non-atherosclerotic vascular pathology that is principally manifested as intermittent claudication because of peripheral vascular ischemia. Precise etiological factors are not yet concretely identified, and it represents 0.1 % of all lower limb claudication causes. Middle-aged males are the most affected gender age group. Misdiagnosis of Popliteal Artery ACD could delay proper management and hence risk the loss of the affected limb due to critical limb ischemia. CASE PRESENTATION: We hereby explore the rare case of a 51-year-old female patient, who complained of vague left lower extremity pain accompanied by paresthesia for 1 month prior to admission without signs of local inflammation. The preoperative radiological assessment suggested the presence of thrombosis within the left Popliteal Artery which caused an occlusion in it and hence the proper blood flow was compromised. CLINICAL DISCUSSION: Surgical intervention and the complete removal of the lesion along with establishing a patent synthetic anastomotic graft to maintain the preexisting vascular bundle was the key to treating our patient. Microscopic analysis of the excised specimen revealed an Adventitial Cystic Disease of the Popliteal Artery. CONCLUSION: Adventitial Cystic Disease represents an extremely rare vascular pathology with a vast margin of non-specific symptoms that could lead to misdiagnoses. It is fundamental to establish suitable preoperative screening protocols for it and sustain adequate levels of clinical awareness so that we can timely diagnose and in turn, achieve proper therapeutic interventions to plummet the potential disastrous complications that could ensue.

An enormous arteriovenous malformation presenting in a child in sacro-gluteal region and managed successfully by recurrent embolisation and surgery.

BACKGROUND: Arteriovenous malformations (AVMs) are rare congenital lesions that affect multiple regions. AVMs often affect the brain and the buttock is the least common. They are usually congenital, but discovered later in life as they are asymptomatic, small and resemble a benign lesion. However, they can abruptly grow and become symptomatic and life-threatening as they can bleed and get infected. PRESENTATION OF CASE: An eight-year-old girl presented with ulcerated and bleeding AVM at the sacro gluteal region. However, due to financial difficulty, it grew to reach an enormous size of (15*15*2 cm) in the buttock. It was later managed by multiple embolisation followed by total surgical resection. DISCUSSION: This case reflects the first case of enormous AVM in the sacro-gluteal region in a child in the Middle East as they rarely reach such huge sizes. Total resection was possible by repeat embolisation and surgery. CONCLUSION: Embolisation can render even huge AVM operable. AVMs should be treated as early as possible as they continue to grow and drastically decrease the quality of life of the patient.