Added value of contrast-enhanced ultrasound (CEUS) in the diagnosis of primary retroperitoneal serous adenocarcinoma: a case report.

BACKGROUND: Primary retroperitoneal serous adenocarcinoma (PRSA) is a rare malignant disease. Given the rarity of the disease, the imaging features of PRSA are unclear. Contrast-enhanced ultrasound (CEUS) also plays an important role in the evaluation of the differential diagnosis of retroperitoneal lesions. CASE PRESENTATION: We report the case of a 62-year-old woman of with increased CA125 levels for 1 year who was referred to our hospital. After conducting contrast-enhanced computed tomography and magnetic resonance imaging, the mass was misdiagnosed as a chocolate cyst. After transvaginal ultrasound (TUS) combined with CEUS, cystadenocarcinoma was considered as the initial diagnosis. Pathology results confirmed PRSA as the final diagnosis. CONCLUSIONS: CEUS features of PRSA are reported for the first time based on this case, potentially aiding in the differential diagnosis of this rare entity before surgery.

PLR and NLR Are Poor Predictors of Survival Outcomes in Sarcomas: A New Perspective From the USSC.

BACKGROUND: Elevations in inflammatory biomarkers, including neutrophil-to-lymphocyte ratio (NLR) or platelet-to-lymphocyte ratio (PLR), are reportedly associated with decreased overall survival (OS) or recurrence-free survival (RFS) in patients with numerous cancers. A large multicenter sarcoma data set was used to determine if elevated NLR or PLR was associated with worse survival and can guide treatment selection. MATERIALS AND METHODS: A total of 409 patients with a primary retroperitoneal sarcoma (n = 268) or truncal (n = 141) sarcoma from 2000 to 2015 were analyzed using the US Sarcoma Collaboration database. Binary NLR and PLR values were developed using receiver operating characteristic curves. Kaplan-Meier model and Cox proportional hazards model identified predictors of decreased OS and RFS. Point biserial analyses were used to correlate binary and continuous data. RESULTS: Neither elevated NLR nor PLR was predictive of decreased OS or RFS. These findings persisted despite exclusion of comorbid inflammatory conditions. Further, NLR and PLR were not correlated with tumor grade. In multivariate models, decreased RFS was associated with tumor factors (e.g., positive margins, tumor grade, tumor size, necrosis, positive nodes); decreased OS was associated with histologic subtype, male gender, and nodal involvement. CONCLUSIONS: Although several small studies have suggested that elevated NLR and PLR are associated with decreased survival in patients with abdominal or truncal sarcoma, this large multicenter study demonstrates no association with decreased OS, decreased RFS, or tumor grade. Rather, survival outcomes are best predicted using previously established tumoral factors.

Non-islet-cell tumour hypoglycaemia (NICTH): About a series of 6 cases.

The purpose of this study was to analyse the characteristics of 6 patients managed in a university hospital between 1996 and 2016 for non-islet cell tumor hypoglycemia (NICTH), a form of hypoglycaemia due to the paraneoplastic secretion of IGF-2 or its related substances. RESULTS: Three of these 6 patients (50%), aged over 69 years, including 2 with acromegaloid phenotype, presented with a pleural solitary fibrous tumor (SFT), with median diameter 20 cm (interquartile range, 12.5-20.5) with a low median SUV (3.3 g/mL (QR, 2-7.5)) on 18F-FDG PET. The other 3 patients presented respectively neuroendocrine carcinoma (NEC) of the palate (70-year-old woman), retroperitoneal myxofibrosarcoma (66-year-old man) and meningeal hemangiopericytoma (36-year-old woman). All 3 were inoperable and did not respond to any therapy other than glucose solution. Corticosteroid therapy was effective in the 3 SFTs and the NEC. One of the SFTs recurred 10 years later with asymptomatic hypoglycemia, which resolved after reintervention. Median (IQR) blood glucose levels of the 6 patients was 0.4g/L (QR, 0.31-0.41), with hypoinsulinemia at 0.7mIU/L (QR 0.7-2.0), undetectable GH, low IGF-1, normal IGF-2 level in 5/6 cases, a high IGF-2:IGF-1 ratio at 26.9 (QR, 20.8-37.8), hypokalemia and hypomagnesemia. CONCLUSION: NICTH is a rare syndrome, which should be considered in the presence of hypoinsulinemic hypoglycemia with low GH and IGF-1, and a IGF-2:IGF-1 ratio>10. Corticosteroid therapy was effective in elderly subjects, particularly with solitary fibrous tumor, which was generally operable. Hemangiopericytoma and myxofibrosarcoma had poor prognosis in younger patients.

Both serum and tissue Galectin-1 levels are associated with adverse clinical features in neuroblastoma.

BACKGROUND: Neuroblastoma is one of the most common pediatric solid tumors. Although the 5-year overall survival rate has increased over the past few decades, high-risk patients still have a poor prognosis due to a lack of biomonitoring therapy. This study was performed to investigate the role of Galectin-1 in neuroblastoma biomonitoring therapy. PROCEDURE: A tissue microarray containing 37 neuroblastoma tissue samples was used to evaluate the correlation between Galectin-1 expression and clinical features. Blood samples were examined to better understand whether serum Galectin-1 (sGalectin-1) could be used for biomonitoring therapy. Kaplan-Meier analysis and ROC analysis was conducted to distinguish the outcome associated with high or low expression of Galectin-1 in patients with neuroblastoma. RESULTS: Increased Galectin-1 expression was found in neuroblastoma and it was further demonstrated that elevated tissue Galectin-1 expression was related to INSS stage, histology, bone marrow metastasis, and poor survival. sGalectin-1 levels were higher in newly diagnosed patients with neuroblastoma than healthy subjects. Patients with elevated sGalectin-1 through treatment cycles correlated with the poor chemo-responses and tended to have worse outcomes, such as metastasis or stable tumor size, whereas gradually decreasing sGalectin-1 levels correlated with no observed progression in clinical symptoms. CONCLUSIONS: Tissue and serum Galectin-1 levels were associated with adverse clinical features in patients with neuroblastoma, and sGalectin-1 could be a potential biomarker for monitoring therapy.

Sirolimus Therapy for Patients With Lymphangioleiomyomatosis Leads to Loss of Chylous Ascites and Circulating LAM Cells.

A young woman received a diagnosis of abdominal, sporadic lymphangioleiomyomatosis (LAM) and multiple abdominal lymphangioleiomyomas and was referred for recurrent chylous ascites responding only to a fat-free diet. On admission, pulmonary function test (PFT) results showed a moderate reduction in the transfer factor for carbon monoxide with normal exercise performance. The serum vascular endothelial growth factor D (VEGF-D) level was 2,209 pg/mL. DNA sequences, amplified at loci kg8, D16S3395, D16S3024, D16S521, and D16S291 on chromosome 16p13.3, showed a loss of heterozygosity (LOH) only for kg8. Fat-free total parenteral nutrition in association with sirolimus (2 mg po daily) was initiated. Serum sirolimus levels were maintained at concentrations between 5 and 15 ng/mL. After 1 month, reintroduction of a low-fat oral feeding was achieved without recurrence of ascites. PFT results were stable. Interestingly, clinical improvement was associated with a reduction in the VEGF-D serum level (1,558 pg/mL). LOH at the kg8 biomarker in blood LAM cells was no longer detected.

Retroperitoneal paravertebral ganglioneuroma: a multidisciplinary approach facilitates less radical surgery.

BACKGROUND: Ganglioneuroma (GN) of the adult is a rare benign tumour originating from neural crest-derived cells. In most cases, GN is found in the mediastinum or retroperitoneum incidentally and may present with unspecific symptoms caused by space-occupying effects. The correct diagnosis of a retroperitoneal mass is still a challenge. Nevertheless, a preoperatively confirmed diagnosis of GN may support the concept of a less radical approach and may help to prevent unnecessary morbidity or loss of function. CASE PRESENTATION: We report a case of a symptomatic retroperitoneal paravertebral GN in a 33-year-old woman. She has been referred with abdominal discomfort, lancinating pain in the right leg, headache and nausea. Magnetic resonance imaging revealed a solid paravertebral tumour adjacent to the psoas muscle. Computed tomography-guided core needle biopsy yielded the diagnosis of GN. The tumour was resected completely via a laparotomy. Immunohistopathological examinations confirmed a benign GN. CONCLUSIONS: Diagnostic studies and therapeutic interventions of retroperitoneal GN are discussed. In our case, a core needle biopsy preceding complete resection was helpful to prevent too extensive surgical approach.

Retroperitoneal Functioning Paraganglioma--A Rare Case of Secondary Diabetes.

INTRODUCTION: Paragangliomas are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia, which can derive from either parasympathetic or sympathetic paraganglia and are closely related to pheochromocytomas. CASE REPORT: We present the case of a young male patient of 37 years old, who was admitted for hypertensive crisis and palpitations. His medical history included medically controlled type 2 diabetes mellitus, (diagnosed 10 months ago), Hepatitis A. Hormonal evaluation revealed elevated urinary metanephrines and normetanephrines, with mainly increased normetanephrines (2330 ug/24 h). Plasmatic metanephrins were in normal range, but levels of plasmatic normetanephrins were elevated (952 pg/ml). The assessment of pituitary and aldosterone-renin axis values were within normal limits. Abdominal computed tomography showed left adrenal nodular lesion on the external arm, bilobulated, size 32/33 mm with maximum axial and cranio-caudal diameter of approx. 45 cm, suggestive of a benign lesion, keeping the cleavage plane to vecinatate structures. Left adrenalectomy was performed by laparoscopic approach. We mention that immediately after induction of anesthesia were recorded blood pressures of 298/143 mmHg. Histopathologic and immunohistochemical examination diagnose paraganglioma, without invasion of adjacent tissues. The patient evolution was favorable, with the remission of the symptoms and normalization of hormonal markers. It is imperative to note the remission of diabetes in the postoperative period. DISCUSSION: This is the case of a young patient with functional retroperitoneal paraganglioma, who presented with symptoms of pheochromocytoma. Compared to pheochromocytomas, paragangliomas are rarely symptomatic and functional. Association with diabetes is even more rare. Specialized investigations allowed the proper diagnosis and the therapeutic approach above was the result of a multidisciplinary cooperation.

[Ectopic production of calcitriol by non-Hodgkin lymphoma as a cause of hypercalcemic crisis]. / Ektope Calcitriol-Produktion durch ein Non-Hodgkin-Lymphom als Ursache einer hyperkalzämischen Krise.

HISTORY AND ADMISSION FINDINGS: A 92-year-old woman was admitted because her general condition had deteriorated during the last two weeks and acute kidney injury had developed. Moreover, she suffered from periods of disorientation and confusion while heretofore she was autonomous. INVESTIGATION, TREATMENT AND COURSE: Clinical and biochemical evaluation revealed a hypercalcemic crisis with markedly increased serum levels of calcium (3.77 mmol/l; reference values 2.2-2.65), an acute kidney injury and neuropsychiatric disturbances. Ultrasound scan and magnetic resonance imaging of a palpable mass in the right abdomen showed a tumor in the retroperitoneal space. Histological evaluation specified the tumor as non-Hodgkin lymphoma. Further investigation revealed no other reason for the hypercalcemia but enhanced levels of calcitriol. Since serum levels of calcidiol were increased while levels of calcidiol were normal, we assumed ectopic production of calcitriol by non-Hodgkin lymphoma as the cause of hypercalcemic crises. This could been proven by the decrease of calcium and calcitriol levels during the course of lymphoma treatment. CONCLUSION: Hypercalcemia of malignancy is the most common cause of hypercalcemia in the inpatient setting. The evaluation of these cases should consider ectopic production of calcitriol a cause of hypercalcemia.

Long-term outcome of vincristine-aspirin-ticlopidine (VAT) therapy for vascular tumors associated with Kasabach-Merritt phenomenon.

BACKGROUND: . This study aimed to clarify the combinatorial treatment effect of agents as aspirin and ticlopidine associated with vincristine in the management of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenic coagulopathy that occurs in the presence of an enlarging vascular tumor such as kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). PROCEDURE: . A retrospective review was conducted of medical records of all children with diagnosis of KHE or TA associated with KMP treated with vincristine-aspirin-ticlopidine (VAT) therapy at two different institutions in the same country from 1994 to 2011. Clinical features, response to VAT therapy and outcomes were recorded. RESULTS: . Eleven patients (mean age 11 months, range 0-36), including seven females (64%) and four males (36%), were identified. Seven patients underwent incisional biopsy and two different histologies were found, KHE in four patients and TA in three patients. Tumors were located in the head and neck (n = 5), chest wall (n = 2), arm (n = 2) and retroperitoneum (n = 2). Mean platelet level was 10,200/mm(3) (range 4,000-21,000). A plaque-like lesion with ecchymosis was the most common cutaneous manifestation (63%). All patients underwent VAT therapy. Mean duration of treatment was 3.9 months for vincristine, 13.9 months for aspirin, and 13.4 months for ticlopidine. All patients are alive with a mean follow-up of 4.5 years (range, 2-17). CONCLUSIONS: . Antiaggregant therapy is helpful in combination with vincristine in the treatment of KMP associated with KHE and TA. Prognosis is excellent if severe thrombocytopenia is controlled despite failure in reduction of tumor size.

Operative considerations in pediatric retroperitoneal teratomas--a review.

BACKGROUND: Retroperitoneal teratomas account for 5% of teratomas and occur predominantly in infants. The resection of these tumors has been variously reported as easy or difficult. This report presents a series of retroperitoneal teratomas and reviews the literature focusing on the relevant operative considerations in this tumor. METHODS: A retrospective chart review from 1996 to 2011 identified five children with retroperitoneal teratomas. Clinical features, radiologic findings, operative details, and outcome were recorded. The literature was reviewed to identify operative complications for these tumors. RESULTS: Out of the five patients, four were infants. The tumor was characterized by dense adhesions in all patients, and resection was complicated by gastric and common bile duct injury. Hiatal hernia repair was required in one and spillage was seen in two patients. One patient had a staged resection after birth with colostomy, vesicostomy, and eventual death on day 12 of life. Inferior vena cava (IVC) ligation was required in one. A total of four patients were well on long-term follow-up. Review of the literature revealed that computed tomography scan while being sufficient for diagnosing a retroperitoneal teratoma is often inadequate in delineating the vascular anatomy, which is usually distorted. The IVC and the mesenteric vessels may be inseparable and require ligation. Renal vessel injury is possible, leading onto ischemia and hypertension. The tumor may be intimately adherent to the viscera in the abdomen, making resection complicated. Adequate resection is possible and necessary for cure, but not always possible with clear resection margins. Late complications include recurrence, adhesions, and hypertension. The perioperative mortality is 0 to 8% and the complication rate is 50 to 100%. CONCLUSION: Surgical resection of retroperitoneal teratomas, while being the mainstay of treatment is challenging in view of its size, adhesions, and vascular distortion.

A retroperitoneal NF1-independent malignant peripheral nerve sheath tumor with elevated serum CA125: case report and discussion.

Malignant peripheral nerve sheath tumors (MPNSTs) are usually located in the trunk, extremities, head, or neck, and most occur with neurofibromatosis type 1 (NF1; von Recklinghausen's disease). No biomarkers have previously been found to be associated with their progression. Retroperitoneal NF1-independent MPNSTs are rare; they are considered to be less aggressive and to have better prognoses compared to NF1-related tumors. Currently, en bloc excision is the only consensus treatment approach. In a 27-year-old male with a giant retroperitoneal MPNST and no stigmata or family history of neurofibromatosis type-1 (NF1), a remarkable elevation of serum CA125 was detected. The high-grade tumor displayed a striking progression: the primary lesion, 25 cm in diameter, recurred in its previous site as a 17-cm MPNST less than 50 days after total excision. Subsequent treatment with microwave ablation and huachansu, a traditional Chinese medication, proved ineffective, and the patient died within 3 months. Our case suggests that retroperitoneal MPNSTs can deteriorate rapidly even if NF1 independent, that aggressive treatment may not benefit large high-grade MPNSTs, and that novel and effective treatment is urgently needed. Our case also suggests the possibility of using serum tumor markers in the early detection and monitoring of MPNSTs.

Malignant adrenal rest tumor of the retroperitoneum producing adrenocortical steroids.

We present a case of a malignant adrenal rest tumor arising from the retroperitoneum with Cushing's syndrome in a 31-year-old female. Her serum cortisol and dehydroepiandrosterone sulfate levels were elevated, while adrenocorticotropic hormone levels were low. Computed tomography scans and magnetic resonance imaging revealed a retroperitoneal tumor with no visible lesions in the adrenal glands and ovaries. From those results and the histopathologic findings following biopsy of an enlarged supraclavicular lymph node, the patient was diagnosed as a malignant adrenal rest tumor of the retroperitoneum. Despite chemotherapy, the patient died of rapid development of multiple metastases. Autopsy revealed a large tumor that extended around the abdominal aorta from the levels of the left kidney to the aortic bifurcation with generalized metastases. Tumor cells were characterized by clear and eosinophilic cytoplasm and atypical nuclei that exhibited frequent and atypical mitoses. Immunohistochemistry regarding steroidogenesis was performed and revealed that the tumor cells were immunopositive for adrenal 4 binding protein/steroidogenic factor-1, cholesterol side-chain cleavage enzyme, 17α-hydroxylase, and 21-hydroxylase. We thus elucidated the adrenocortical steroid production in the tumor cells causing Cushing's syndrome. This case report first demonstrates the steroidogenic capacity in a malignant adrenal rest tumor.

[Management of a retroperitoneal paraganglioma]. / Prise en charge d'un paragangliome rétropéritonéal.

Paragangliomas are rare tumors arising from extraadrenal chromaffin cells. We report a 43-year-old man who presented with abdominal pain. An abdominal computed tomography scan revealed a large retroperitoneal mass. During an endoscopic biopsy of this tumor, the patient experienced marked hemodynamic fluctuations with tachycardia and high blood pressure, and an extraadrenal pheochromocytoma was suspected. Measurements of plasma and urinary catecholamines and urinary total metanephrines ruled in the diagnosis. Echocardiography disclosed acute myocardial dysfunction that returned to normal after surgical resection of the paraganglioma. This report also underlines the importance of the anesthetic preparation and monitoring around the surgical procedure and the need of a long-term follow-up to detect malignant paraganglioma in the absence of histological criteria of benign tumor.

Retroperitoneal solitary fibrous tumor-induced hypoglycemia associated with high molecular weight insulin-like growth factor II.

A man, aged 65 years, presented with frequent episodes of hypoglycemia and unconsciousness. Hypoglycemia was accompanied by undetectable serum insulin and C-peptide levels and a high serum insulin-like growth factor (IGF)-II level. He was found to have a retroperitoneal solitary fibrous tumor. He underwent successful resection of the tumor and had no hypoglycemic episodes after the operation. Immunohistochemical analysis revealed positive immunostaining for IGF-II in tumor cells. The presence of the high-molecular-weight form of IGF-II in the patient's serum was confirmed by immunoblotting, which suggests that his hypoglycemia was due to an increase in the plasma level of IGF-II secreted by the tumor.

American Society of Clinical Oncology Clinical Practice Guideline on uses of serum tumor markers in adult males with germ cell tumors.

PURPOSE: To provide recommendations on appropriate uses for serum markers of germ cell tumors (GCTs). METHODS: Searches of MEDLINE and EMBASE identified relevant studies published in English. Primary outcomes included marker accuracy to predict the impact of decisions on outcomes. Secondary outcomes included proportions of patients with elevated markers and statistical tests of elevations as prognostic factors. An expert panel developed consensus guidelines based on data from 82 reports. RESULTS: No studies directly compared outcomes of decisions with versus without marker assays. The search identified few prospective studies and no randomized controlled trials; most were retrospective series. Lacking data on primary outcomes, most Panel recommendations are based on secondary outcomes (relapse rates and time to relapse). RECOMMENDATIONS: The Panel recommended against using markers to screen for GCTs, to decide whether orchiectomy is indicated, or to select treatment for patients with cancer of unknown primary. To stage patients with testicular nonseminomas, the Panel recommended measuring three markers (alpha-fetoprotein [AFP], human chorionic gonadotropin [hCG], and lactate dehydrogenase [LDH]) before and after orchiectomy and before chemotherapy for those with extragonadal nonseminomas. They also recommended measuring AFP and hCG shortly before retroperitoneal lymph node dissection and at the start of each chemotherapy cycle for nonseminoma, and periodically to monitor for relapse. The Panel recommended measuring postorchiectomy hCG and LDH for patients with seminoma and preorchiectomy elevations. They recommended against using markers to guide or monitor treatment for seminoma or to detect relapse in those treated for stage I. However, they recommended measuring hCG and AFP to monitor for relapse in patients treated for advanced seminoma.

[Exploring . . . a retroperitoneal mass in children]. / Comment j'explore ... une masse retroperitoneale chez l'enfant.

Detection of a retroperitoneal mass in children needs a fast and accurate exploration. Wilms tumor and neuroblastoma, the most frequent, will be presented more in detail including their clinical and biological characteristics, their diagnostic tests and their primary therapeutic treatments.

Mujer de 38 años con poliglobulia y una masa abdomina / A 38-year-old woman with polyglobulia and a abdominal mass

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