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INTRODUCTION AND IMPORTANCE: Carney complex (CNC) is an extremely infrequent multiple endocrine neoplasia syndrome characterized by distinctive pigmented skin and mucosal lesions, cardiac and noncardiac myxomatous tumors, and multiple endocrine tumors. We herein report a case of CNC and surgical and history of laparoscopic left adrenalectomy complicated with a primary pigmented nodular adrenocortical disease (PPNAD). PRESENTATION OF CASE: We present the case of a 38-year-old woman with a previous diagnosis of CNC and history of laparoscopic left adrenalectomy who consulted for severe depression refractory to medical treatment. In the laboratory tests performed, altered ACTH, prolactin, Somatomedin C-IGF-1 and estradiol. An abdomen and pelvis C/T scan was requested, where an 8 mm lesion was found at the level of the right adrenal gland. Laparoscopic right adrenalectomy was performed. Histopathology of the surgical resection specimen revealed PPNAD. DISCUSSION: CNC is an infrequent syndrome with autosomal dominant inheritance and genetically heterogeneous. PPNAD is a consistent feature in CNC patients, however, reports of Cushing's syndrome in the literature indicate that only 25-45 % of CNC patients have PPNAD. CONCLUSION: PPNAD can be present in patients with Carney complex, with surgical adrenalectomy history. With an adequate selection of patients, laparoscopic adrenalectomy with subsequent hormone replacement therapy should be performed.
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INTRODUCTION AND IMPORTANCE: The solitary fibrous tumor (SFT) is a rare tumor of mesenchymal origin, with a reported incidence of 2.8 cases per 100,000 tumors and with distinctive histopathological and immunohistochemical characteristics. It was initially described as a pleural lesion and subsequently, it was found in different organs and tissues. The abdominoinguinal incision described by Karakousis allows a safe and radical approach for lower quadrants abdominopelvic tumors. CASE PRESENTATION: A 47-year-old man was referred to us with a 5-months history of lower backache radiating to the left lower limb. MRI and CT revealed a retroperitoneal mass of 10 cm extending to left iliac vessels. The initial diagnosis corresponded to a sarcomatous retroperitoneal tumor. It was decided to perform an abdominal exploration using Karakousis's approach for surgical resection. The immunohistochemistry and histopathological study revealed neoplasia compatible with a SFT. It was categorized as low risk for developing metastasis and death from disease, according to the new malignancy criteria. Currently, the patient is asymptomatic and disease-free at 19 months after surgery. CLINICAL DISCUSSION: Most patients with SFTs present symptoms derived from the tumor growth and the compression on adjacent structures with clinical manifestations that are frequently insidious and precede the tumor discovery. The diagnosis is based on histopathological studies. Nonetheless, when they present an extrathoracic location, they represent a diagnostic challenge, due to their variable histological characteristics. CONCLUSION: Presacral SFT is a rare entity, with a scant incidence reported regarding this location and long-term treatment. Surgical resection is needed as the immediate treatment.
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Cecal endometriosis is a rare entity that can present as nonspecific acute abdominal pain and can be complicated by ileocolic intussusception, which is extremely infrequent. We present the case of a 33-year-old woman with no relevant pathological antecedents who consulted for abdominal pain for 5 days, associated with rebound tenderness and abdominal guarding on the right lower quadrant and a palpable mass during the physical examination. Computed tomography was realized and emergency surgery performed due to suspected ileocolic intussusception. The laparoscopic examination identified an ileocolic intussusception associated with a tumor. Conversion to open surgery was needed, and an oncological right hemicolectomy with ileotransverse anastomosis was carried out. Histopathological study reported ileocolic intussusception and a focus of cecal endometriosis. Currently, the patient does not have recurrences. Ileocolic intussusception secondary to deep endometriosis requires great diagnostic presumption in women of childbearing age with acute abdomen diagnosis.
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El paciente con multimorbilidad crónica forma parte de una población que se ha incrementado en los últimos años. La hernia inguinal incarcerada representa una emergencia. El abordaje transabdominal preperitoneal (TAPP) presenta ventajas para evaluar el contenido de la hernia. Se presenta el caso de un hombre de 77 años con múltiples comorbilidades y cirugías abdominales previas, que se presentó con cuadro de oclusión intestinal. En tomografía computada: asa de intestino delgado incarcerada en región inguinal. Se observó asas intestinales dilatadas con cambio de calibre en región inguinal izquierda. Se retiró asa intestinal atascada en hernia directa. Se disecó defecto herniario y se colocó malla de 10 x 15 cm en espacio preperitoneal. La técnica TAPP es eficaz y segura para la reparación de hernias complicadas en pacientes con multimorbilidad crónica, en manos de cirujanos experimentados.
The patient with chronic multimorbidity is part of a population that has increased in recent years. Incarcerated inguinal hernia represents an emer-gency. The preperitoneal transabdominal approach (TAPP) has advantages to evaluate the content of the hernia. A 77-years-old man with multiple comorbidities and previous abdominal surgeries presented with intestinal occlusion. Computed tomography: small bowel loop incarcerated in the inguinal region. The cavity is inspected by observing dilated intestinal loops with a change of caliber in the left inguinal region. The intestinal loop is removed observing a direct hernia. The hernia defect is repaired, and 10 x 15 cm mesh is placed in the preperitoneal space. The TAPP te-chnique is effective and safe for the repair of complicated hernias in patients with chronic multimorbidity, in the hands of experienced surgeons.
Assuntos
Humanos , Masculino , Idoso , Laparoscopia , Multimorbidade , Hérnia Inguinal , Relatos de Casos , Doença CrônicaRESUMO
El páncreas anular (PA) es una anomalía congénita infrecuente. Se caracteriza por la presencia de tejido pancreático ectópico alrededor del duodeno y puede estar asociado a obstrucción duodenal. Se presenta un paciente con cuadro de dolor abdominal y vómitos de 12 días de evolución. El laboratorio mostró elevación de lipasa en sangre. La tomografia computarizada de abdomen evidenció estómago y primera porción duodenal distendidos, en relación con una imagen en anillo de 5 cm de diámetro ubicada entre la cabeza del páncreas y la segunda porción del duodeno, sugestiva de PA. Ante la mala respuesta al tratamiento médico con reposo digestivo, sonda nasogástrica y nutrición parenteral, se decide conducta quirúrgica confirmando el diagnóstico de obstrucción duodenal por un PA. Se realiza gastroyeyunostomía en Y de Roux con buena evolución posquirúrgica. El PA puede manifestarse clínicamente como una obstrucción duodenal. Los casos con mala respuesta al tratamiento conservador requieren conducta quirúrgica para confirmar el diagnóstico y resolver la oclusión.
Annular pancreas (AP) is a rare congenital anomaly, characterized by ectopic pancreatic tissue surroun-ding the duodenum, that may associate with duodenal obstructon. We present a patent complaining of 12 days of abdominal pain and vomitis. Blood testis showed hyper-lipasemia. Computed tomography scan demonstrated stomach and frst duodenal porton distended in relaton to a 5 cm diameter image resembling a ring between the pancreatic head and the second porton of the duodenum, a well known characteristic of AP. Following a lack of clinical response to medical treatment, surgery was performed confirming a duodenal obstructon due to AP. Roux-en-Y gastrojejunostomy was done, with good postoperative outicome. Annular pancreas may present as a duodenal obstructon. For these cases, we suggest surgical treatment to confirm diagnosis and resolve the obstructon in cases with negative clinical response to conservative treatment.