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PURPOSE: To analyze the role of laparoscopic surgery for the management of pediatric adrenal tumors (AT). METHODS: Retrospective analysis of children diagnosed with AT, operated laparoscopically during 2003-2020. The strategy differed according to tumor extension. AT < 6 cm were resected. Locally advanced tumors (L2) or > 6 cm were biopsied. RESULTS: N = 28. Complete tumor resection (R0) in 20 (71%), tumor biopsy in 8 (R2). Age (median): 28.8 months (2 months-18 years). 14/28 left-sided, 2 bilateral. Median operating time: 78 min (45-180). Mean tumor size (for resections): 4 cm (2.5-6). Tumor pathology: neuroblastoma (n = 17), Ganglioneuroma (n = 7), Adrenocortical carcinoma (n = 1), Osteosarcoma metastasis (n = 1), Pheochromocytoma (n = 1), Venous malformation (n = 1). Mean hospital stay: 2.5 days (1-3). Mean follow up: 65.5 months (24-192). Overall survival and event-free survival were 86 and 75%, respectively (5 years event-free survival for neuroblastoma: 33% [intermediate risk], 16.6% [high risk]. No surgery-related mortality. CONCLUSION: Laparoscopic surgery for adrenal tumors is safe. Laparoscopic biopsy is useful for unresectable tumors when a percutaneous approach is not possible. With the proposed selection criteria, the laparoscopic approach should be the first option for resection of small and localized AT in pediatrics.

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RESUMEN Introducción: Las causas adrenales del síndrome de Cushing como el carcinoma son las etiologías comunes en el niño, si bien su incidencia es baja. Objetivo: Describir los procedimientos diagnósticos y terapéuticos seguidos en el caso de una lactante de cinco meses con síndrome de Cushing como consecuencia de un carcinoma adrenocortical. Presentación del caso: Lactante femenina de cinco meses consultada por aumento excesivo de peso a partir del segundo mes de nacida a pesar de no alimentarse adecuadamente, además de presentar un retraso en el crecimiento. Se constató obesidad centrípeta y facie pletórica, ligero eritema facial y livedo reticularis, tensión arterial de 130/80 mmHg (>95 percentil). Presentaba agenesia de labios menores, sin vello axilar o pubiano. En el estudio de la función suprarrenal se encontró cifras elevadas de cortisol plasmático. En la tomografía computarizada abdominal se observó imagen hiperdensa compatible con tumor suprarrenal. Se diagnosticó síndrome de Cushing de etiología suprarrenal. Se le realizó adrenalectomía unilateral y se tomó muestra para biopsia posoperatoria y se emitió el diagnóstico de carcinoma adrenocortical. La paciente presenta una evolución desfavorable y finalmente fallece a consecuencia de un fallo multiorgánico al mes de realizada la cirugía. Conclusiones: Ante la sospecha clínica de síndrome de Cushing en la lactante se realizaron las determinaciones hormonales pertinentes y precisó la etiología mediante pruebas de imagen, confirmada por biopsia posquirúrgica. La cirugía constituye la terapia más adecuada ante un paciente con síndrome de Cushing de causa tumoral adrenal, aunque estos pacientes por lo general cursan con mal pronóstico.

ABSTRACT Introduction: The adrenal causes of Cushing's syndrome such as carcinoma are the common etiologies in the child, although their incidence is low. Objective: Describe the diagnostic and therapeutic procedures followed in the case of a five-month-old infant with Cushing's syndrome as a result of adrenocortical carcinoma. Case presentation: Five-month-old female infant consulted due to excessive weight gain from the second month of birth despite not eating properly, in addition to presenting a stunted growth. Centripetal and facial plethora obesity, slight facial erythema and livedo reticularis, blood pressure of 130/80 mmHg (>95 percentile) were found. She had agenesis of labia minora, without axillary or pubic hair. In the study of adrenal function, elevated plasma cortisol levels were found. Abdominal CT scan showed hyperdense image compatible with adrenal tumor. Cushing's syndrome of adrenal etiology was diagnosed. Unilateral adrenalectomy was performed and a sample was taken for postoperative biopsy and the diagnosis of adrenocortical carcinoma was made. The patient presented an unfavorable evolution and she finally died as a result of a multi-organ failure a month after the surgery. Conclusions: Given the clinical suspicion of Cushing's syndrome in the infant, the pertinent hormonal determinations were made and the etiology was specified by imaging tests, confirmed by post-surgical biopsy. Surgery is the most appropriate therapy for a patient with Cushing's syndrome with an adrenal tumor cause, although these patients usually have a bad prognosis.

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BACKGROUND: Genitourinary tract tumors in children are less common than in adults. Most of these tumors have different genetic backgrounds, clinical presentation, and oncologic behavior than their adult counterpart. As a result of low prevalence in children, some of the treatment approaches and recommendations are based on treatment experience in adult patients. However, thanks to scientific and technological development, survival rates have risen considerably. OBJECTIVE: This paper presents a review of the principal features of the tumors involving the genitourinary tract in children and an update in genetic background, diagnosis, and treatment. METHODS: A narrative review was performed on published literature about genitourinary tract tumors in pediatric patients. Papers presented in English and Spanish literature were reviewed. PubMed, Science Direct, and SciELO databases were used to collect information and present this article. RESULTS: Kidney tumors are the most common type of genitourinary tumors in children. Among those, Wilms tumor represents the majority of cases and shows the successful work of clinical trial groups studying this tumor type. Other tumors involving the genitourinary tract in children include Rhabdomyosarcoma, Transitional cell carcinoma, Testicular, and Adrenal tumors. CONCLUSION: Genitourinary tract tumors in children represent significant morbidity and economic burden, so awareness in early diagnosis represents improvement in treatment, clinical, and oncological outcomes.

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Primary neuroendocrine differentiation in prostate cancer occurs infrequently and represents a therapeutic challenge at present due to the poor prognosis involved. We present the case of a patient with de novo neuroendocrine prostate cancer who later developed metastases to adrenals bilaterally which were initially managed surgically.

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CASE SUMMARY: This case report describes a 9-year-old neutered male cat with a functional adrenal tumor showing aggression toward other cats in the household, excessive meowing and urine spraying. The diagnosis was made based on the clinical signs, an enlarged right adrenal gland on ultrasound, penile spines and elevated sex hormones on an adrenocorticotropic hormone stimulation test. The cat was submitted to celiotomy and adrenalectomy. Histopathological examination confirmed the adrenocortical adenoma. Three months after surgery the penile spines disappeared and urine spraying, as well as excessive meowing, had greatly decreased; however, aggressive behavior took much longer to stop and required behavior therapy. RELEVANCE AND NOVEL INFORMATION: Functional adrenal tumors producing only sex hormones and behavioral changes are uncommon. The cat reported herein started showing behavioral changes before spines appeared on the penis, and structural alterations in the adrenal gland at the abdominal ultrasonography were detected. Considering all the possible implications resulting from severe behavior problems such as cat-cat aggression, from a permanent separation of the cats to relinquishment, a detailed investigation of underlying medical mechanisms in these animals is crucial from the start. Examinations may need to be repeated later in the course.

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Adrenocortical cancer is a rare malignant neoplasm associated with a dismal prognosis. Identification of the molecular pathways involved in adrenal tumorigenesis is essential for a better understanding of the disease mechanism and improvement of its treatment. The aim of this study is to define the prevalence of alterations in DNA mismatch repair (MMR) genes in Lynch syndrome among pediatric patients with adrenocortical neoplasia from southern Brazil, where the prevalence of a specific TP53 germline mutation (p.Arg337His) is quite high. Thirty-six pediatric patients were retrospectively evaluated. Immunohistochemistry (IHC) for the MMR enzymes MLH1, MSH2, MSH6, and PMS2, as well as next-generation sequencing (NGS) were performed. For IHC, 36 pediatric tumors were tested. In all of them, the expression of all evaluated MMR proteins was well-preserved. For NGS, 35 patients with pediatric tumor were tested. Three patients (8.57%) with the TP53 p.Arg337His germline mutation presented pathogenic and likely pathogenic variants in the MMR genes (two in MLH1 and one in MSH6). The prevalence of altered MMR genes among pediatric patients was elevated (8.57%) and higher than in colorectal and endometrial cancer cohorts. Pediatric patients with adrenocortical tumors should, thus, be strongly considered as at genetic risk for Lynch syndrome.

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Background: Ganglioneuroma is a rare tumor derived from the neural crest that can occur in any sympathetic tissue. It corresponds to 0.3% to 2% of incidental adrenal tumors and <250 have been reported in the literature so far. Case Presentation: We present a case of a 30-year-old Caucasian woman presented with a large bilobed adrenal tumor found on a CT scan during the investigation of acute abdominal pain. The image also showed a rare anatomic variation of a left-sided inferior vena cava. Biochemical work-up for adrenal incidentaloma showed normal markers. Since we could not rule out malignancy, the patient was subjected to laparoscopic adrenalectomy and the pathology report showed an adrenal ganglioneuroma, a rare nonfunctioning tumor of the adrenal. Conclusion: Ganglioneuroma can present as a large bilobed adrenal tumor. The laparoscopic approach is feasible and safe. Preoperative planning is needed and vascular variations can be challenging during the procedure.

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Background: Adrenal incidentalomas are masses accidentally discovered during imaging examinations performed whenthere is no suspicion of adrenal disease. Even with a low prevalence, it is important to perform a reliable evaluation observing biological behavior and determining whether the hormonal activity is stimulated. Frequently, these masses are notfunctional, but in some cases, there is increased cortisol activity, and patients with adrenal incidentalomas may presenthyperadrenocorticism. This report aims to describe the clinical, tomographic, and histopathological aspects of a case ofadrenal incidentaloma detected in a routine abdominal ultrasound.Case: An 8-year-old, male, maltese dog with occasional emesis, halitosis, claudication of the left pelvic limb, and previous compensated and untreated cardiomyopathy was treated at the Veterinary Hospital of the Federal University of MatoGrosso (HOVET-UFMT). Physical examination revealed arterial hypertension and claudication, with the positive posteriordrawer test suggesting rupture of the cruciate ligament. The blood count showed no alteration, and the serum biochemistryrevealed a slight increase in the activity of alanine aminotransferase. In order to investigate this increase, an abdominalultrasound was performed. Slight hepatomegaly and a heterogeneous mass of irregular edges were observed with a moderate and difficult-to-delimit vascularization of the adrenal gland. Left knee radiography raised the suspicion of rupture ofthe cranial cruciate ligament due to the cranial displacement of the tibia in relation to the femur. Computed tomographywas performed to define the extent and delimitation of the mass, which was compatible with a tumor of the right adrenalgland characterized by the visualization of an abdominal mass...(AU)

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El oncocitoma suprarrenal es un tumor extremadamente infrecuente, con apenas unos 150 casos descriptos. Paciente de sexo masculino, de 48 años, con adrenalectomía derecha por incidentaloma suprarrenal de 7 cm, no funcionante. El oncocitoma suprarrenal no puede distinguirse desde el punto de vista clínico-imagenológico de otros tumores adrenales más frecuentes, por lo que su confirmación es histológica. Rara vez son malignos, y el tratamiento aceptado es la suprarrenalectomía, idealmente por vía laparoscópica. En nuestro caso, la presencia de dos criterios histológicos menores lo clasifican como un tumor con potencial maligno.

Oncocytic adrenocortical carcinoma is an extremely rare tumor, with only 150 cases reported in the literature. A 48-year-old male patient, with right adrenalectomy due to a 7-cm non-functional adrenal incidentaloma. From a clinical imaging point of view, oncocytic adrenocortical carcinoma cannot be distinguished from other more common adrenal tumors; therefore, the diagnostic confirmation is histological. It is rarely malignant, and the accepted treatment is laparoscopic adrenalectomy. In our case, two minor histological criteria classify it as a potentially malignant tumor.

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Ectopic adrenal gland is an atypical medical condition with a prevalence of less than 1%. It occurs due to an abnormal gland development during embryonic life and can be found in different parts of the body. Rarely, these ectopic glands can be hormonally active and present as bulky masses. Herein we report a case of a patient who underwent laparoscopic surgery for a large myelolipoma associated with an androgen producing adenoma in an ectopic adrenal gland.