Laparoscopic right adrenalectomy in a patient with Carney syndrome: A case report.

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.

Surgical resection of a presacral solitary fibrous tumor with extension to iliac vessels using Karakousis's abdominoinguinal approach: Report of a rare case.

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.

Ileocecal intussusception as the initial presentation of endometriosis: case report.

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.

Metachronous extragastrointestinal stromal tumor (EGIST) in utero: Report of an unusual case.

INTRODUCTION: The gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor in the digestive tract. Currently, GIST is the name given to CD117 positive mesenchymal tumors, primary of the digestive tract, mesentery, and retroperitoneum. Nevertheless, they have been reported in the mesentery, omentum, gallbladder, bladder wall, and few cases in the uterus; known as extragastrointestinal stromal tumors (EGIST). PRESENTATION OF CASE: Seventy-six-year-old woman with a history of the third recurrence of pelvic tumor located in the uterus initially diagnosed as uterine leiomyosarcoma. CT and MRI showed a tumor in the uterine corpus of approximately 10 cm. It was decided to perform the surgical rescue. The immunohistochemistry and anatomic pathology report revealed a tumor compatible with a uterine EGIST. It was decided to perform adjuvant treatment with imatinib. Currently, the patient continues to be disease-free 20 months after the surgery. DISCUSSION: For years, GIST has often been confused with leiomyosarcoma, given that they are histologically almost indistinguishable. The IHC analysis for KIT (CD117) has become essential in the GIST diagnosis. On the other hand, stromal tumors arising outside the gastrointestinal tract are rare (5%), which have a histological and biological behavior similar to that of GISTs. CONCLUSION: EGISTs are extremely rare and often incidentally detected. Currently, evidence about this location is scarce. According to the literature, this is the first case of uterine EGIST with a metachronous presentation.

[Congenital renal arteriovenous fistula: a rare cause of acute abdomen and a review of its literature]. / Fístula arteriovenosa renal congénita: una causa rara de abdomen agudo y revisión de la literatura.

Introduction: Congenital renal arteriovenous fistula is an abnormal connection between the arterial and venous system. Since the first case described in 1928 by Varela et al, no more than 200 cases have been published. Material and methods: A 45-year-old woman consulted for severe abdominal and low-back pain associated with arterial hypertension. The CT scan showed an infrarenal retroperitoneal lesion with invasion of neighboring structures. Results: During the abdominal exploration, a vascular-looking lesion of 60 x 34 x 41 mm was identified. It was associated with right nephrectomy. Discussion: The etiology of congenital fistulas remains unknown; it is believed that a congenital arterial aneurysm erodes into an adjacent vein and gradually increases its size. Other authors believe that the fistula exists since birth and gradually increases its size until it causes symptoms. Conclusion: Congenital arteriovenous fistulas are rare entities, representing less than 25% of all renal arteriovenous malformations.

Introducción: La fístula arteriovenosa renal congénita es una conexión anómala entre el sistema arterial y venoso. Desde el primer caso descrito en 1928 por Varela y cols, no más de 200 casos han sido publicados. Material y Método: Mujer de 45 años consultó por dolor abdominal y lumbar severo, asociado a hipertensión arterial. En tomografía computada se observó lesión retroperitoneal infrarrenal con invasión de estructuras vecinas. Resultados: En exploración abdominal se identificó lesión de aspecto vascular de 60 x 34 x 41 mm. Se asoció a nefrectomía derecha. Discusión: La etiología de las fístulas congénitas sigue siendo desconocida, se cree que un aneurisma arterial congénito erosiona hacia una vena adyacente y aumenta su tamaño lentamente. Otros autores creen que la fístula existe desde el nacimiento e incrementa progresivamente de tamaño hasta producir síntomas. Conclusión: Las fístulas arteriovenosas congénitas son entidades raras, representando menos del 25% del total de las malformaciones arteriovenosas renales.

Técnica mínimamente invasiva en paciente con multimorbilidad crónica y hernia inguinal complicada: reporte de caso / A minimally invasive technique in a patient with chronic multimorbidity and complicated inguinal hernia: case report

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.

Multiple intestinal perforations due to primary mediastinal B-cell lymphoma: A case with an infrequent extra-nodal presentation.

INTRODUCTION: Primary mediastinal B-cell lymphoma (PMBCL) is an uncommon subtype of non-Hodgkin lymphoma (2-3%), predominantly occurring in female young adults. Extrathoracic involvement is found in 10-20%. It can affect the kidneys, pancreas, stomach, adrenal glands, liver, and infrequently the central nervous system (6-9%). There is currently only one reported case of ileum dissemination with a single perforation. PRESENTATION OF CASE: A 51-year-old woman with a history of PMBCL, hospitalized by a superior vena cava syndrome. PET-CT showed numerous lesions in the small intestine, pancreas, adrenal glands, and left kidney. During chemotherapy she presented abdominal symptoms, requiring an emergency laparotomy. On examination, six perforation sites were found in the small intestine. The pathology report revealed lesions compatible with PMBCL spread. DISCUSSION: There are few case series with reports of dissemination in the gastrointestinal tract, with the main location in the stomach. Knowing the visceral location of the PMBCL would allow us to plan a strict follow-up during the first phases of chemotherapy treatment, as well as the early diagnosis of unexpected complications, such as intestinal perforation. CONCLUSION: The PMBCL is a rare entity. Visceral involvement should be suspected in these patients since intestinal perforation represents a complication with high morbidity and mortality. This is the first case reported with numerous intestinal locations and multiple post-chemotherapy perforations.

Hemicolectomía derecha laparoscópica con abordaje suprapúbico: una técnica novedosa para el tratamiento del cáncer de colon / Suprapubic laparoscopic right hemicolectomy: a novel technique for the treatment of colon cancer

Antecedentes: la hemicolectomía derecha laparoscópica con abordaje suprapúbico (HDLS) y empleo de tecnología robótica o laparoscópica de incisión única ha sido recientemente informada. La utilización de la técnica estándar multipuerto en HDLS no se ha descripto previamente. Material y métodos: entre enero y agosto del año 2018 fueron intervenidos 4 pacientes, 3 mujeres y 1 hombre, con mediana de 64 años de edad y diagnóstico de adenocarcinoma de colon derecho. Resultados: el procedimiento se realizó exitosamente en todos los pacientes, con una mediana de tiempo operatorio de 210 minutos (r:170-240). Ningún paciente tuvo complicaciones y fueron dados de alta en una mediana de 4 días (r:3-5). Todas las piezas quirúrgicas tuvieron márgenes negativos y un recuento ganglionar > 12 ganglios. A 7, 5, 4 y 2 meses del seguimiento, los pacientes se hallan vivos y libres de enfermedad. Conclusión: la técnica de HDLS multipuerto es una alternativa sencilla, factible y segura para el tratamiento del cáncer de colon en pacientes seleccionados, con un resultado funcional, estético y oncológico favorable.

Background: The suprapubic approach for laparoscopic right hemicolectomy has been reported with robotic surgery or single incision laparoscopy. The use of the suprapubic approach for standard multiport laparoscopic right hemicolectomy has not been previously described. Material and methods: Between January and September 2018, four consecutive patients (three women and one man; median age: 64 years) with right-sided colon cancer underwent laparoscopic right hemicolectomy using the suprapubic multiport approach. Results: The procedure was successful in all the patients and mean operative time was 210 minutes (IQR: 170-240). There were no complications and were discharged on postoperative day 4 (IQR: 3-5). All the surgical specimens had negative margins and lymph node count was > 12 lymph nodes. All the patients are alive and free from disease at 7, 5, 4 and 2 months of follow-up. Conclusion: The suprapubic approach for standard multiport laparoscopic right hemicolectomy is an easy, feasible and safe alternative for the treatment of colon cancer in selected patients, with a favorable functional, esthetic and oncological result.

Laparoscopic Distal Pancreatectomy With Peritoneal Staple-line Reinforcement: A Novel Technique (With Video).

INTRODUCTION: Postoperative pancreatic fistula (PPF) is still the major source of morbidity in distal pancreatectomy (DP). Despite the many surgical techniques and technology devices developed for the closure of the pancreatic stump, the PPF rate remains high, and there is no consensus with regard to the most appropriate stump closure technique. We herein present, for the first time, an innovative approach for staple-line reinforcement in DP using an autologous peritoneal patch. MATERIALS AND METHODS: The results of 2 consecutive patients who underwent laparoscopic DP due to mucinous cystic neoplasms were included in this report. The pancreas was transected with a reinforced stapler using a peritoneal patch obtained from the anterolateral wall of the abdomen, divided into 2 pieces, and sutured around each stapler jaw. The embedded video (Supplemental Digital Content 1, http://links.lww.com/SLE/A209) reports our standardized technique. RESULTS: Both patients had a favorable recovery without PPF or any complication and were discharged fully recovered 4 and 5 days after surgery without drains. Currently, both patients are alive and free of disease 1 year and 8 months after the procedure. CONCLUSIONS: The use of an autologous parietal peritoneal patch for staple-line reinforcement in DP was feasible and safe in experienced hands. Further studies are needed to confirm our findings and elucidate whether this readily available, inexpensive, rapid, and versatile alternative could reduce the PPF rate.

Multiple primary tumors: Colorectal carcinoma and non-Hodgkin's lymphoma.

INTRODUCTION: Colorectal cancer (CRC) is the third most commonly diagnosed cancer, whereas lymphoma is the sixth leading cause of cancer death, 90% of which corresponds to non-Hodgkin's lymphoma (NHL). The association of these two primary tumors, a solid tumor with an hematological malignancy, is very uncommon. PRESENTATION OF CASE: We report the case of a 47-year-old man who presented with abdominal pain, a right upper quadrant mass and 12 kg of weight loss in 9 months. The computed tomography (CT) showed a large intra-abdominal mass and a wall thickening at the rectosigmoid junction. A colonoscopic biopsy confirmed a colorectal adenocarcinoma and a laparoscopic biopsy of the intraabdominal mass confirmed a diffuse large B-cell NHL. After multidisciplinary discussion it was decided to treat first the NHL with 3 cycles of R-CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone). The patient experienced a good response with a 70% decrease in the intraabdominal mass and a negative PET/CT. Four months after diagnosis an anterior rectal resection was performed. The patient recovered uneventfully and was discharged 5 days after surgery. The patient finally died 20 months after surgery due to disease progression. DISCUSSION: The association of CRC and NHL is an extremely rare scenario that represents a great multidisciplinary challenge with respect to treatment due to the scarce literature found on this topic. CONCLUSION: When CRC and NHL are present, all the different disease patterns must be considered in a multidisciplinary and patient-oriented fashion, in order to decide the best therapeutic strategy for each individual.

Obstrucción duodenal por páncreas anular / Duodenal obstructon due to anular pancreas

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.

Malignant fibrous histiocytoma in the right portion of the mandible with metastasis in pancreas.

BACKGROUND: Malignant fibrous histiocytoma is a sarcoma of uncertain origin that can be found both in soft tissues and in bones. It is currently called undifferentiated pleomorphic sarcoma not otherwise specified and it represents a final common pathway in several tumors that are subject to the progress of dedifferentiation. Local recurrence of the tumor in the same location where it was originated occurs in 20-30% of the total number of soft tissue sarcomas. It is less frequent in limbs and more likely to recur in retroperitoneal sarcomas and in head and neck. Most tumors grow during the first two years after treatment. Most sarcoma metastases, especially those in the head and neck (including malignant fibrous histiocytoma) present as a lung disease (90%). Extrapulmonary diseases are not frequent and can occur in lymph nodes (10%), bones (8%) and in the liver (1%). CASE REPORT: 61 years old woman with history of malignant fibrous histiocytoma in the right portion of the mandible, resected four years ago. Subsequent checkups did not show any disease. The patient reports a constant abdominal pain not diagnosed, related to a pancreatic nodule. CONCLUSION: Malignant fibrous histiocytoma is a potentially curable disease. The most important part of the treatment is complete surgical removal, usually followed by adjuvant radiation therapy. Chemotherapy is a treatment usually used only on those patients with higher recurrence risk or on those showingrecurrence or who have already showed recurrence.