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BACKGROUND: Pediatric intracranial germ cell tumors (iGCT) are rare, with limited data available from Arabic countries. METHODS: We retrospectively reviewed the medical charts of children <18 years diagnosed with iGCT at King Hussein Cancer Center/Jordan (January 2003 to December 2020) for clinical characteristics, treatment, and morbidities. RESULTS: Sixteen patients had germinoma; median age was 6.9 years and median symptoms duration 8 months. Nine tumors were suprasellar, five pineal, and two bifocal. Four were metastatic. Eight patients had slightly elevated beta subunit human chorionic gonadotropin and 11 patients had resection/biopsy. Fifteen patients received chemotherapy; mostly carboplatin (450 mg/m2 )/etoposide, which had low toxicity. All patients received radiotherapy (different doses and fields). At median follow-up of 7.7 years, one tumor recurred (progression-free survival: 91% ± 8%). Twelve patients who continued follow-up had stable visual and endocrine deficits to their initial presentation. Five finished or are finishing diploma and seven had poor school performance (four left school). Six patients were diagnosed with nongerminomatous germ cell tumor; median symptom duration was 1 month. Three tumors were pineal, two suprasellar, and one at quadrigeminal plate. Three were metastatic. Five tested patients had high tumor markers and four had resection/biopsy. All patients received chemotherapy, and then five received craniospinal radiation. Two patients are alive, two died with tumor progression, one died in remission with electrolyte imbalance, and one developed leukemia and died with septic shock. CONCLUSIONS: We achieved excellent survival in treating germinoma using a feasible protocol for low middle-income countries. However, patients encountered significant morbidities exacerbated by delayed diagnosis and unnecessary surgical interventions despite abnormal tumor markers. Raising awareness on iGCT symptomatology and diagnosis may help limit these morbidities.
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Neoplasias Encefálicas , Germinoma , Neoplasias Embrionárias de Células Germinativas , Criança , Masculino , Humanos , Jordânia/epidemiologia , Estudos Retrospectivos , Estudos de Viabilidade , Germinoma/patologia , Neoplasias Embrionárias de Células Germinativas/tratamento farmacológico , Neoplasias Encefálicas/terapia , Neoplasias Encefálicas/tratamento farmacológico , Biomarcadores Tumorais , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêuticoRESUMO
INTRODUCTION: Ligation of the renal pedicle is a crucial step in laparoscopic renal harvesting. Currently, two devices are widely used: Hem-o-Lok® clips and staplers. Each has its advantages and disadvantages. METHODS: A systematic literature review was conducted following the PRISMA methodology on PubMed, EMBASE, and Cochrane databases with no time restrictions. Registered in the PROSPERO database (CRD42023460585), the study's primary outcome measures include the failure rate of the ligation tool, conversion to open surgery, rate of severe bleeding, and device-related death. Other secondary variables are also explored. An assessment of the quality of included comparative studies was performed using the Risk Of Bias In Non-randomised Studies of Interventions (ROBINS-I) tool. RESULTS: Out of 393 articles identified using our keywords, 32 were included. Overall, clips have a lower failure rate during application, elongate graft vessel length, and are more cost-effective. Staples are associated with a shorter warm ischemia time and lower blood loss. There is no significant difference between the two devices in terms of conversion, severe bleeding, death, and blood transfusion. CONCLUSION: There is no perfect modality for renal pedicle control during laparoscopic harvesting, as each instrument has its pros and cons. Hem-o-Lok® clips are a safe and cost-effective option, particularly suitable for right-sided harvesting or in cases of vascular variations. However, they are still associated with a risk of secondary displacement with potentially serious consequences. Certain technical tricks can mitigate this risk.
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Transplante de Rim , Laparoscopia , Doadores Vivos , Nefrectomia , Instrumentos Cirúrgicos , Humanos , Nefrectomia/métodos , Laparoscopia/métodos , Laparoscopia/instrumentação , Laparoscopia/efeitos adversos , Transplante de Rim/métodos , Coleta de Tecidos e Órgãos/métodos , Coleta de Tecidos e Órgãos/instrumentação , Grampeamento Cirúrgico/métodos , Grampeamento Cirúrgico/instrumentação , Perda Sanguínea Cirúrgica/prevenção & controleRESUMO
INTRODUCTION: Thyroid follicular renal cell carcinoma is a special type of renal cell carcinoma newly recognized in recent years. The data is not mature due to the rarity of cases. The association of vesicular papillary tumors of the thyroid is exceptional, and this is the first publication describing such an association in the literature. CASE PRESENTATION: We present the case of a patient who consulted for a goiter. The definitive pathological examination of the specimen of the thyroidectomy showed papillary vesicular thyroid tumors. A month later, she presented with total hematuria; the CT scan revealed a left renal mass; the patient underwent a partial nephrectomy; and the definitive pathological examination of the specimen showed a thyroid follicular carcinoma-like renal tumor. DISCUSSION: Thyroid-type follicular cell renal cell carcinomas are currently recognized as a distinct entity whose histological appearance is reminiscent of thyroid vesicular lesions. There are currently around 39 cases in the literature, but no concomitant thyroid localization has been observed. This finding cannot be verified in the absence of a systematic histological study of the thyroid gland. Our case invites discussion of other thyroid investigation modalities, in particular the value of thyroid biopsy versus cytopuncture, which is often inconclusive in this type of situation. CONCLUSION: At present, understanding of TFCLRT is still very limited. Even more so, their association with a thyroid tumor is exceptional in the literature. We need to increase the number of cases and conduct in-depth investigations with longer follow-up periods to better understand the situation.
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Emphysematous pyelonephritis and cystitis, rare and potentially life-threatening infections marked by gas accumulation in the urinary tract, are described in a 32-year-old male with a history of myelomeningocele and a neurogenic bladder managed by a Benchekroune valve. Clinical and radiological findings confirmed emphysematous pyelonephritis and cystitis. A conservative approach, including urinary drainage and four-week antibiotic treatment (ceftriaxone and metronidazole), led to rapid clinical improvement. This case underscores the successful management of emphysematous infections in a neurogenic bladder patient, emphasizing the effectiveness of conservative treatment.
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This case details a 42-year-old farmer experiencing ten months of lower abdominal pain, diagnosed with a retrovesical hydatid cyst (RVHC). Imaging, including ultrasound and CT scans, categorized the cyst as Gharbi type 3. Serological tests confirmed echinococcal antibodies. Surgical intervention comprised the resection of the cyst's prominent dome, following one month of albendazole therapy. The importance of a multidisciplinary approach is highlighted, emphasizing collaboration among urologists, radiologists, and parasitologists for optimal outcomes. This case discussion contributes to the existing literature, providing insights into the management of this rare manifestation of echinococcosis.
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INTRODUCTION AND IMPORTANCE: Advances in medical imaging have led to increased detection of rare adrenal cysts, typically asymptomatic. The article emphasizes their silent nature and the evolving diagnostic methods. CASE PRESENTATION: Woman underwent successful laparoscopic removal of a 9 cm left adrenal cyst. Histological analysis identified it as an endothelial cyst. CLINICAL DISCUSSION: Adrenal cysts, mostly unilateral, have diverse histological types. Diagnosis relies on imaging, and surgical intervention is required for symptomatic cases or those exceeding 5 cm. Minimally invasive techniques have improved outcomes. CONCLUSIONS: Adrenal cysts, generally benign and asymptomatic, pose diagnostic challenges. Surgical intervention is recommended for larger or symptomatic lesions, with advancements in minimally invasive options improving outcomes.
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Peripheral neuroectodermal tumors (PNET), or Ewing sarcoma, are tumors that generally develop in bone; extraskeletal locations are rare. Renal PNETs are rare and are characterized by an aggressive clinical course and a poor prognosis. We report the case of a young patient who presented with abdominal and lumbar pain with a palpable abdominal mass. The imaging was in favor of a huge renal tumor, and the histological analysis allowed the diagnosis of a renal PNET. The therapeutic attitude was multimodal, including surgery and chemotherapy, allowing complete remission and a favorable outcome.
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INTRODUCTION AND IMPORTANCE: This case report describes the successful use of a latissimus dorsi muscle flap attached to the femoral vessels to cover a large mass loss in a 27-year-old woman with bladder exstrophy. The two-stage reconstruction showed excellent results six months after surgery. CASE PRESENTATION: A 27-year-old woman with bladder exstrophy and previous enterocystoplasty with Mitrofanoff-type urinary diversion combined with a late fascial flap presented 10 years later with disembowelment near the pubic symphysis. A two-stage reconstruction using a latissimus dorsi flap and polypropylene mesh was performed with excellent aesthetic and functional results. CLINICAL DISCUSSION: The latissimus dorsi muscle flap, described by J.M. Servant in 1984 as the "apple turnover" technique, is highly reliable for reconstructing large substance losses with minimal functional sequelae. The procedure in this case resulted in very satisfactory aesthetic and functional results at six months postoperatively. This approach provided a safe and effective technique of last resort. CONCLUSION: the two-stage latissimus dorsi flap technique was a reliable, safe and effective solution for this complex reconstructive challenge.
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INTRODUCTION: Renal cell carcinoma is one of the 10 most common malignancies. Partial nephrectomy (PN) is the preferred treatment for localized ones. The incidence of local recurrence (LR) after a PN varies between zero and 17% in the literature. Managing a LR following PN is a challenging situation, both ablation therapy and salvage surgery are viable options. CASE PRESENTATION: A 38-year-old woman with a history of a right open partial nephrectomy performed on an ectopic kidney four years ago, presented a loco regional recurrence, involving the right ovary and rectus muscle, which we managed by a radical nephrectomy with bilateral oophorectomy et resection of the rectus muscle nodules. CLINICAL DISCUSSION: LR of RCC after PN presents a clinically challenging scenario. Treatment options include ablative therapy or surgical salvage therapy, which can be either by radical nephrectomy or by repeat partial nephrectomy. Ablative therapy has less complication rate and is preferred for small LR or on a solitary kidney. Repeat PN is also an option for preserving renal function but with high risk of complication. RN is an option if PN is technically challenging, especially in patients with a functional contralateral kidney. CONCLUSION: Recurrent renal cell carcinoma (RCC) can manifest differently based on the primary treatment, as well as tumor and patient characteristics. Managing a LR following PN is a challenging situation, both ablation therapy and salvage surgery are viable options.
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INTRODUCTION: Bowen's disease (BD) represents an in situ squamous cell carcinoma that can progress to an invasive one without treatment. Various options for Bowen's disease have been delineated, each with its set of advantages and disadvantages. CASE PRESENTATION: We report the case of a 60-year-old patient with a history of chronic smoking and a background of multiple partners and recurrent urethritis. The patient presented with a maculopapular lesion on the lateral aspect of the penis, evolving for 5 years. A biopsy confirmed the diagnosis of Bowen's disease. The patient underwent an excision of the lesion which the histology showed an infiltrating basosquamous cell carcinoma. DISCUSSION: The diagnosis of Bowen's disease requires a biopsy and is based on histological examination. Only surgical treatment allows for the identification, through histological analysis of the excised specimen, of any potential invasive area that may not have been identified in the biopsy. Nonsurgical therapies are also an option with high recurrence rates. CONCLUSION: Bowen's disease management requires a personalized approach, considering factors like lesion characteristics, patient-related variables and treatment efficacy. An adapted follow-up is recommended due to the recurrence risk associated with various treatments.
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Although germ cell tumours can appear in childhood, they are most common around the age of 30. These tumours are highly responsive to chemotherapy, and even cases of relapse have relatively high cure rates. There is limited literature on patients diagnosed after the age of 50, and no specific trials have been carried out in this context. These patients, considered 'elderly', are treated with the same cisplatin-containing combinations as younger patients, despite the higher toxicity. This report presents an observation of a giant testicular mixed germ cell tumour discovered in a 75-year-old patient.
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An intravesical ureterocele is a rare condition in which a terminal ureter terminates in a cystic dilation of the bladder. We present the case of a 42-year-old female who presented with irritative lower urinary tract symptoms and left lower back pain. Computed tomography (CT) urography revealed ureteral duplication with a ureterocele complicated by upper tract obstruction. Treatment involved endoscopic ureterocelotomy, which successfully relieved symptoms and resolved renal obstruction.
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Ureter , Obstrução Ureteral , Ureterocele , Feminino , Humanos , Adulto , Ureter/cirurgia , Ureterocele/complicações , Ureterocele/diagnóstico , Ureterocele/cirurgia , Obstrução Ureteral/etiologia , Pelve Renal , EndoscopiaRESUMO
INTRODUCTION: Renal cell carcinoma (RCC) occasionally involves the inferior vena cava (IVC), but intra-atrial extension is very rare, with the most dreaded complication being thrombi migration into pulmonary arteries. CASE PRESENTATION: A 52-year-old male with rheumatoid arthritis and a smoking history presented with dyspnea, weight loss, and a previous episode of hematuria. Echocardiography revealed an intra-atrial thrombus with signs of pulmonary embolism (PE). Subsequent evaluation found a massive right renal tumor with an extensive thrombus extending into the right atrium, causing PE. Multidisciplinary management included surgery involving nephrectomy and thrombectomy via cavotomy and open-heart surgery along with pre- and postoperative anticoagulation. DISCUSSION: Intra-caval tumor thrombus (TT) in RCC is infrequent, especially when extending into the right atrium. Accurate diagnosis involves imaging modalities, with echocardiography and MRI playing pivotal roles. Multidisciplinary and personalized management is mandatory to ensure a successful outcome. Surgery remains the primary treatment for RCC with TT but carries significant risks. CONCLUSION: Managing RCC with cavo-atrial tumor thrombus is a complex situation that requires a collaborative approach. Accurate staging and tailored treatment are crucial. Surgical intervention remains pivotal in the absence of alternative effective modalities. Ongoing research into adjuvant therapies is crucial for optimal outcomes.
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Peritoneal fistulization of pyonephrosis is an uncommon condition, leading to a generalized peritonitis, considered as extreme life-threatening emergency. Secondary to an obstructive uropathy, increasing intra-renal pressure. In most cases, ureteral stones represent the underlying causes followed by stenosis, tumor, or connective tissue disease. Through this case report, we present a 60-year-old patient with a history of urolithiasis, admitted for an atypical instance of spontaneous renal pelvis rupture in an ectopic right kidney managed surgically. This case emphasizes the importance of considering renal etiology in cases of peritonitis and underscores the rarity of such occurrences.
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Penoscrotal lymphedema is a manifestation of disrupted lymphatic drainage, causing a significant increase in scrotal volume. impacting both aesthetics and quality of life. Elephantiasis, classified as stage III by the International Society of Lymphology, represents the advanced stage of scrotal lymphedema, often linked to parasitic diseases. The diagnosis is clinical, and the treatment involves mass excision. The lymphatic reconstruction is an innovative therapeutic approach that improves postoperative results and better quality of life. In this case report, we present the case of massive genital elephantiasis managed surgically, and we will focus on surgical techniques and reconstructive approaches.
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Wünderlich's syndrome, a rare and life-threatening condition involving spontaneous renal hemorrhage, is commonly associated with renal angiomyolipoma (rAML). Management options for rAML include surveillance, mTOR inhibitors, selective arterial embolization (SAE), and surgery. When complicated by Wünderlich's syndrome, prompt and adequate interventional treatment is necessary to prevent fatal outcomes. We present a unique case of a young female patient with Tuberous Sclerosis Complex who experienced a massive ruptured rAML, leading to Wünderlich's syndrome. Our approach involved a multidisciplinary hybrid strategy, combining SAE and nephrectomy, highlighting SAE's valuable role even when radical treatment is required.
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Angiomiolipoma , Embolização Terapêutica , Neoplasias Renais , Esclerose Tuberosa , Feminino , Humanos , Neoplasias Renais/complicações , Angiomiolipoma/complicações , Angiomiolipoma/terapia , Rim , Esclerose Tuberosa/complicaçõesRESUMO
Embryonal rhabdomyosarcoma of the prostate in adults is rare and often diagnosed at an advanced stage, with metastases. We report the case of a 23-year-old young adult presenting with low back pain and dysuria, whose imaging revealed a voluminous metastatic prostate mass. Biopsy confirmed embryonal rhabdomyosarcoma. Treatment was initiated with chemotherapy, resulting in significant regression of the tumour mass and metastases after 3 courses. Pediatric advances suggest improved survival with a multimodal approach, but its efficacy in adults requires further investigation.
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Bladder injuries, although rare, can occur as a complication of hip surgery, particularly when a hip prosthesis migrates into the bladder. We present the case of a 75-year-old woman with a bladder rupture secondary to prosthesis migration requiring repair via a transvesical approach. While total hip arthroplasty (THA) is common, intrapelvic complications such as bladder injury are less commonly reported. Early recognition and appropriate treatment are essential to avoid serious consequences. The management of prosthesis migration into the bladder is complex and requires detailed anatomical knowledge. Awareness of this potential complication is essential for both orthopaedic surgeons and urologists.
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Tubular ectasia of the rete testis (TERT) presents as multiple cystic structures within the rete testis, often incidentally detected on ultrasound as echo-free intratesticular cystic lesions. Despite its benign nature, assessing testicular cystic lesions can sometimes be challenging. The primary importance of identifying this uncommon condition lies in its distinction from cystic testicular malignancies and thus avoiding further radical procedures. We report an instance of TERT within the right testis discovered incidentally in a patient with a medical background of epididymitis, presenting for management of left testis cryptorchidism, and bilateral inguinal hernia.
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Post-traumatic hematoma is often associated with severe, extensive injuries. In patients with blood coagulation disorders, hematoma can occur even after innocuous injuries. Among these coagulation disorders is hemophilia, an X-linked recessive disorder characterized by hemorrhagic manifestations. The aim of this article is to report a case of massive scrotal hematoma following trauma in a 24-year-old hemophiliac with no signs of severity who benefited from medical treatment with infusion of coagulation factors with a good clinico-biological evolution.