Retroperitoneal cystic lymphangioma in a cirrhotic patient: A case report and review of the literature.

Retroperitoneal cystic lymphangiomas (RCL) are rare benign tumors of the lymphatic system. They account for less than 1% of all lymphangiomas. Surgical resection is the recommended treatment option; however, obtaining a pre-operative diagnosis is often difficult and, in most cases, the final diagnosis is only possible following histological assessment of the surgical specimen. This report describes a case of RCL in a 58-year-old female cirrhotic patient who presented to our center with dull aching abdominal pain and distension. To our knowledge, this is the first case of a RCL in a cirrhotic patient reported in the literature.

Is Hypoglycemia Secondary to Metastatic Retroperitoneal Sarcoma - A Therapeutic Challenge? Case Report and Review of Literature.

Most soft tissue sarcomas afflict the extremities; however, the retro peritoneum can also be affected rarely. Retroperitoneal sarcomas are relatively asymptomatic. Although tumor-induced hypoglycemia is rare in tumors other than insulinomas, extrapancreatic tumors are a subset that displays this phenomenon. The occurrence of hypo-insulinemic hypoglycemia with low GH and IGF-1 should prompt consideration of the secretion of a hypoglycemic substance impeding the secretion of insulin and GH, such as IGF-2 or one of its related substances. The present case report is of a 38-year-old male with retroperitoneal round cell sarcoma with liver metastasis with severe symptomatic hypoglycemia who was managed with multipronged symptomatic therapy and oncological management after which he had shown significant improvement in hypoglycemic episodes and symptom profile. A literature review revealed our case report to be the first reported case of a young male (preponderance in the older population) with hypoglycemia associated with retroperitoneal sarcoma which presented with liver metastasis and the only one treated with Gemcitabine /Docetaxel. The presence of these features might point toward a poorer prognosis in a disease with an already dismal course. All these points towards the need for further research regarding intensified oncological treatment after evidence-based prognostication of high-risk groups and modalities for the management of symptomatic hypoglycemia such as Somatostatin analogs and glucagon which aid in symptom control.

Secreting retroperitoneal latero-aortic paraganglioma revealed by acute abdominal pain: a case report.

We report the case of a retroperitoneal paraganglioma in a 35-year-old man discovered by abdominal pain. The abdomino-pelvic computed tomography (CT) showed a retro-peritoneal latero-aortic mass compatible with a paraganglioma, confirmed by the 24-hour urinary metanephrines and normetanephrines test. After an adequate pharmacological preparation, we decide to perform a laparoscopic resection of the paraganglioma. The surgery went without any complication. Blood pressure and urinary catecholamines were normal after the surgery. Our observation presents the particularity of the mode of discovery which is atypical, as well as the particularity of the therapeutic management, which is the laparoscopic resection of the mass.

Sarcopenia and frailty as predictors of surgical morbidity and oncologic outcomes in retroperitoneal sarcoma.

BACKGROUND: Retroperitoneal sarcomas (RPS) are rare tumors for which surgical resection is the principal treatment. There is no established model to predict perioperative risks for RPS. We evaluated the association between preoperative sarcopenia, frailty, and hypoalbuminemia with surgical and oncological outcomes. METHODS: We performed a prospective cohort analysis of 65 RPS patients who underwent surgical resection. Sarcopenia was defined as Total Psoas Area Index ≤ 1st quintile by sex. Frailty was estimated using the modified frailty index (mFI). Logistic regression models were used to assess predictors of 30-day postoperative morbidity. The Kaplan-Meier method with log-rank test was utilized to assess factors associated with overall (OS) and recurrence-free survival (RFS). RESULT: Sarcopenia was associated with worse OS with a median of 54 compared with 158 months (p = 0.04), but no differences in RFS (p > 0.05). Hypoalbuminemia was associated with worse OS with a median of 72 compared with 158 months (p < 0.01). MFI scores were not associated with OS or RFS (p > 0.05). Sarcopenia, mFI, and hypoalbuminemia were not associated with postoperative morbidity (p > 0.05). CONCLUSION: This study suggests that sarcopenia may be utilized as a measure of overall fitness, rather than a cancer-specific risk, and the mFI is a poor predictive measure of outcomes in RPS.

Central diabetes insipidus with anti-rabphilin-3A antibody positivity causing hypovolemic shock after resection of tumorous lesions in the pelvic cavity.

A 36-year-old female was pointed out to have liver enzyme elevation by routine health checkup. Subsequent contrast-enhanced CT scan identified gigantic uterine fibroids and retroperitoneal tumor. She was referred to the gynecologist at JA Toride Medical Center and planned to undergo a uterus enucleation and biopsy of the retroperitoneal tumor. The surgery was conducted without any troubles. After the surgery, the patient presented polyuria with urine volume 10-20 L a day and developed hypovolemic shock. Laboratory test revealed hypotonic urine and hypernatremia. Arginine vasopressin (AVP) loading test suggested shortage of endogenous vasopressin. Since the subcutaneous administration of AVP was not sufficient to control the urine volume, continuous intravenous infusion of AVP was initiated. After achieving hemodynamic stability, the treatment was switched to oral desmopressin. MRI finding indicated attenuation of high signal in posterior pituitary in T1 weighted image while neither enlargement of pituitary nor thickening of pituitary stalk was indicated by enhanced MRI. Hypertonic salt solution test indicated no responsive elevation of AVP, confirming the diagnosis of central diabetes insipidus (CDI). Her anterior pituitary function was preserved. Only anti-rabphilin-3A antibody was found positive in the serum of the patient, while other secondary causes for CDI were denied serologically and radiologically. Hence, lymphocytic infundibuloneurohypophysitis　(LINH) was suspected as the final diagnosis. Hormonal replacement therapy by nasal desmopressin was continued and the patient managed to control her urine volume. In cases of CDI considered idiopathic with conventional examinations, anti-rabphilin-3A antibody may be a clue for determining the cause as LINH.

Sarcopenia is associated with worse surgical complications but not relapse-free survival and overall survival in patients with retroperitoneal liposarcoma.

OBJECTIVE: This investigation aimed to explore the relationship between sarcopenia and severe postoperative complications, relapse-free survival (RFS), and overall survival (OS) in patients with retroperitoneal liposarcoma (RLPS). MATERIAL AND METHODS: This retrospective study included 72 RLPS patients (47 men, 25 women; mean age, 57.49 years, SD 10.92) who had abdominal CT exams. Clinical information was recorded, including RLPS characteristics (histologic subtypes, grade, size), laboratory assessment (ALB, PALB, A/G, Hb, SCr), relapse-free survival, overall survival, and postoperative complications. The relationships between those variables and RFS and OS were analyzed using Cox proportional hazard models. RESULTS: There were 8 severe postoperative complications (Clavien-Dindo grade > 2). The chi-square test showed sarcopenia was associated with severe postoperative complications (P = 0.011). In multivariate analysis, sarcopenia was not associated with relapse-free survival (P = 0.574) and overall survival (P = 0.578). CONCLUSIONS: Sarcopenia predicts worse surgical complications but does not affect relapse-free survival and overall survival.

Complex Surgical Management of Radiation-Associated Left Retroperitoneal Sarcoma.

Radiation-associated sarcomas (RASs) are rare entities that tend to have an aggressive course and poor prognosis. Criteria for diagnosis of radiation-associated sarcoma include therapeutic radiation preceding the development of sarcoma, sarcoma arising within or near the irradiated field, and tumor histology that is distinct from the primary tumor necessitating radiation. Despite their relatively uncommon occurrence, RASs are a well-established complication of radiation therapy. We present the complex, multidisciplinary surgical management of a patient with multi-compartmental radiation-associated sarcoma of the left retroperitoneum occurring nearly 25 years after undergoing whole trunk radiation for Hodgkin's lymphoma.

An asymptomatic giant extra-renal retroperitoneal angiomyolipoma: Case report.

RATIONALE: Angiomyolipoma is a benign mesenchymal tumor that consists of adipose tissue, muscle cells and blood vessel. Renal angiomyolipomas represent almost one percent of all renal tumors. PATIENT CONCERNS: We reported a case of a 42-year-old woman complaining of mild abdominal pain with no other symptoms and no remarkable medical history. DIAGNOSIS: Clinical examination was inconclusive and revealed a large, smooth, non-tender, and immovable mass in the right abdomen. Ultrasound examination confirmed the existence of a large, homogeneous, hyperechoic tissue mass. Abdominal multi-slice computed tomography (CT) scans also confirmed the presence of a well-rounded mass in the right abdomen. The histopathology tests confirmed the diagnosis of a large retroperitoneal mass. INTERVENTIONS: The patient underwent a traditional laparotomy without complications to remove the tumor. OUTCOMES: The open surgery was the best option, and the patient's condition improved due to the following-up. LESSONS: Retroperitoneal extra-renal angiomyolipomas are extremely rare, and in this case, we document a case of retroperitoneal angiomyolipoma manifested with only mild abdominal pain in Syrian women.

Retroperitoneal ganglioneuroma with nodal involvement in an adult patient with human immunodeficiency virus: a case report and review of the literature.

BACKGROUND: Ganglioneuromas are a benign tumor originating from neural crest cells. As one of the neuroblastic tumors, ganglioneuromas are most common in children, with a mean age at presentation of 7 years. Ganglioneuromas are typically singular in nature, but rarely can present with lymph node involvement and distant metastasis. We present a rare case of a retroperitoneal ganglioneuroma found in a human immunodeficiency virus positive adult, which was complicated by lymph node involvement. This case is notable not only in regard to the age of the patient, but also because of his human immunodeficiency virus positive status and the extension of tumor to lymph nodes. CASE PRESENTATION: A 27-year-old Latino male with history of human immunodeficiency virus presented with a 6-month history of left upper quadrant and epigastric abdominal pain with associated nausea and vomiting. The patient had a computed tomography scan showing a retroperitoneal mass encasing the aorta, celiac axis, and superior mesenteric artery. Core needle biopsy revealed ganglioneuroma. Owing to obstructive symptoms, resection of the mass along with partial gastric resection, partial pancreatic resection, and splenectomy was performed by a multidisciplinary group of surgeons. Pathology results revealed metastatic spread of ganglioneuroma to surrounding lymph nodes. CONCLUSIONS: Ganglioneuromas are most common in children and very rarely occur in adults. However, it is still important to consider this entity in the differential for patients presenting with suspicious symptoms. While rare, it is essential to consider metastasis in this generally benign disease.

Giant Myxoid Spindle Cell Neoplasm Presenting with Abdominal Swelling and Concurrent Weight Loss.

Retroperitoneal sarcomas may grow extremely large before becoming clinically symptomatic, and curative resection often has high associated morbidity. An 83-year-old man presented with insidious-onset abdominal pain and weight loss owing to a 16.3 × 13.1 × 25.8-cm retroperitoneal high-grade myxoid spindle cell sarcoma. The patient was ultimately deemed to be unfit for surgery before rapidly succumbing to his disease. This case illustrates both the indolent growth seen in these lesions and the importance of proper patient selection. Older patients with large, high-grade lesions and multiple associated comorbidities are often poor surgical candidates, as the associated surgical morbidity outweighs the potential survival benefit. While the ultimate treatment plan relies on shared decision making by the patient and the healthcare provider, the decision to pursue surgical intervention should take into consideration the patient's broader clinical condition.

Retroperitoneal extension of massive ulcerated testicular seminoma through the inguinal canal: A case report.

INTRODUCTION: Testicular cancers represent about 5% of all urological malignancies and 1-1.5% of all male neoplasms. Most of the testicular cancers are localized (68%) at diagnosis. Bulky masses in the scrotum are rare. We present a rare case of bulky testicular cancer with retroperitoneal spread through the inguinal canal. CASE REPORT: A 44-year-old man came to the emergency department referring weakness and the presence of a scrotal mass. At physical examination, a voluminous mass was found, with necrotic phenomena within the scrotum. Abdomen was tense and sore. Abdominal CT scan revealed a bulky testicular mass spreading to the retroperitoneal space through the inguinal canal with node enlargement. Patient underwent orchiectomy with excision of infiltrated scrotum skin. Histologic diagnosis confirmed a typical form seminoma. The patient was then treated with a cisplatin-based chemotherapy, with a partial response. The patient recently relapsed and he is being treated with a new line of chemotherapy and subsequent surgery with or without radiotherapy. CONCLUSIONS: We described a rare presentation of testicular cancer. This case highlights the importance of a multidisciplinary approach to rare testis tumour presentation and early diagnosis for testicular cancers.

Severe headache presenting as an early sign of retroperitoneal para aortic paraganglioma.

BACKGROUND: Paraganglioma is a rare type of neuroendocrine tumor with the ability to secrete neuropeptide and catecholamines in excess. Sympathetic hyperactivity, severe persistent headache and hypertension is the most common clinical presentation of paraganglioma similar to pheocromocytoma. Case report We reported a case of 19 year old girl with severe headache and hypertension, from past 6 month. On further imaging evaluation for the headache, the computed tomography of the abdomen plus pelvis was suggestive of left pre para aortic paraganglioma measuring of 3.4 cm in diameter. Surgical excision of mass was done. Histopathological examination of surgical specimen was consistent with the diagnosis of paraganglioma. Patient is on regular follow up without any subjective or objective evidence of the disease. CONCLUSION: Retroperitoneal paraganglioma may be one of the causes for commonly occurring symptomatic headache, which is benign in nature, but possibility of transformation of malignant paraganglioma can occur. The surgical excision of mass is the treatment of choice.

Changes of the hepatic subcapsular blood flow in a case of high-grade retroperitoneal liposarcoma: what to expect?

Changes of the hepatic subcapsular blood flow with the early appearance of hypervascularity near the falciform ligament are rare radiologic findings. They present most frequently in cases of superior vena cava (SVC) obstruction and are related to the recruitment of the cavo-mammary-phrenic-hepatic-capsule-portal and the cavo-superficial-umbilical-portal pathways. We present the case of a 52-year-old female patient with an highly aggressive retroperitoneal liposarcoma with SVC obstruction caused by external compression due to a mediastinal metastatic mass. The patient exhibited no symptoms of SVC obstruction due to the collateral cavo-portal pathways.

Resection of a giant retroperitoneal lipoma herniating through the inguinal canal.

Retroperitoneal lipomas are extremely rare with few cases reported so far in the literature. They can reach different sizes and present with a variety of symptoms. The differential diagnosis is mainly with well-differentiated liposarcoma (WDLPS). We present a 34-year-old woman with a retroperitoneal lipoma herniating through the inguinal canal into the proximal thigh. The patient underwent complete oncological resection using a Karakousis's abdominoinguinal incision. Retroperitoneal lipomas are a very rare condition and sometimes require resections technically challenging. MDM2 amplification is critical for its differential diagnosis with WDLPS.

Linfangioma quístico gigante pancreático

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