Conglomerated Lymph Nodes Research Articles

Adalimumab-Inducing Reactivation of Tuberculosis With Previously Negative QuantiFERON Test

Intestinal tuberculosis (TB) is a very rare disease in the 21st Century. This case highlights the necessity of including TB enteritis as a differential diagnosis in patients who had previously negative interferon-gamma release assay (Quantiferron) who presents with non-specific abdominal symptoms while on immunosuppressive therapy. A 37 year old Bangladeshi male with a remote history of treated pulmonary TB and Psoriasis, presented with fever and loose stools for one month. Fever was intermittent, associated with night sweats and mild RLQ discomfort. He denied sick contacts or recent travel history. Three months prior to admission, dermatology had initiated Adalimumab (ADA) for refractory Psoriasis. Prior to starting ADA, sputum and interferon-gamma release assay was negative. Physical exam was notable for temperature of 103 degrees F and mild RLQ tenderness without a discrete mass. CBC, serum transaminases, lipase, viral hepatitis panel, and HIV assay were all negative. ESR was elevated at 110 and CRP at 220. Sputum culture for AFB, stool culture and blood culture were negative. Chest x-ray and CT chest was unremarkable. CT abdomen and pelvis showed short segment mural thickening of the terminal ileum with bowel distention, right retroperitoneal lymphadenopathy and 4.2 cm right retroperitoneal lymph node conglomerate. Pt underwent CT guided biopsy of mesenteric lymph node which showed patchy necrosis, multinucleated giant cells, few granulomas and rare acid fast bacilli. Pt was started on TB therapy with improvement in symptoms. Extra-pulmonary TB (EPTB) is found in approximately 20% of the patients with TB in the U.S. The 6th most common extra pulmonary site is the ileocecal region. Clinical and radiological findings of GI TB often mimic diseases such as Crohn's disease and malignancy. Therefore, this can lead to delays in accurate diagnosis and treatment. This is particularly true for a case like ours, where a prior negative Quantiferron may ‘rule-out' TB as a diagnostic possibility. Our case points out that under circumstances where a high index of suspicion for EPTB exists, particularly in patients with negative PPD, Quantiferron, and sputum, lymph node biopsy can be a very important supplement to our diagnostic armamentarium. An algorithm of these diagnostic methods leads to considerably higher precision in the diagnosis of this insidious disease which primarily necessitates a clinical awareness of this serious health problem.FigureFigure

Double fenestration of the ipsilateral internal jugular vein

To the editor, Internal Juguler vein (IJV) is the largest venous structure in the neck and drains venous blood from cranium, facial region, and the neck. IJV is an important anatomical landmark during surgical and interventional radiological procedures, and a thorough knowledge of its anatomical variations and neighborhood relationships is of paramount significance. Fenestration is a rare malformation of IJV (1-3). A 3-year-old female child was referred by an outside center to our center for computed tomography examination for further evaluation of a mass lesion in her neck. Her physical examination was not remarkable for any abnormality. The laboratory studies (including blood cell count, hemoglobin concentration, and hematocrit) were all normal. A contrast-enhanced multislice computed tomography (MSCT) examination was performed using a 16-slice computed tomography device (Brilliance, Philips, Amsterdam, Netherlands). The tomographic sections showed a tortuous and dilated IJV on the right side of the neck; three dimensional (3D) volumetric images were reconstructed using the axial plane images stored on the workstation. A 3D MSCT angiography showed double fenestration in the right IJV, and dilation and tortuosity in its terminal segment ( Figures. 1A, B ). Additionally, multiple conglomerated lymph nodes were present in bilateral posterior cervical region. The patient was informed about her vascular anomaly and discharged. Fenestrations and duplications of IJV are rare congenital vascular anomalies of neck. However, there is an important difference between fenestration and duplication. The main difference between these two clinical entities is that in duplication IJV originates as a single vessel from the cranium, branches off into two superiorly, and converges distally with the subclavian vein as two separate vessels, whereas in separation the two veins converge with each other before they converge with the subclavian artery, as in our patient (2, 4-5). The incidence of duplication is 0.4% while fenestration has an incidence of less than 1%. These two variations may co-occur with aneurysm and flebectasia. Our patient also had flebectasia and tortuosity in the terminal part of the right IJV. The embryological origin of IJV fenestration is unclear. Vascular, neural, and bony hypotheses have been put forth to explain this variation. Whereas it has been suggested that it occurs at 3-6. gestational weeks, it has also been reported to occur due to clinical causes such as interventional procedures (2-4). IJV fenestrations are usually asymptomatic and usually detected incidentally, as is the case for our patient. However, it is important to show the anatomy of IJV and the accessory nerve during neck dissection in patients with head-neck tumor. The relationship between the accessory nerve and IJV should be clarified in order to protect both structures during neck dissection (1, 3-5). Modern MSCT technology currently allows the visualization of the supra-aortic arterial and venous structures with a high image quality. The obtained images are reformatted and reconstructed in different planes and used to guide planning of endovascular and surgical treatment (3-5). In conclusion, it is of particular importance to have knowledge of this type of anomaly before contemplating surgical and interventional procedures involving the neck region.

Paraneoplastic acral vascular syndrome in a patient with metastatic melanoma under immune checkpoint blockade

BackgroundParaneoplastic acral vascular syndrome (PAVS) is a rare phenomenon which is observed in patients with adenocarcinomas and other malignancies. Various potential pathogenic mechanisms such as tumour invasion of sympathetic nerves, hyperviscosity, hypercoagulability, vasoactive tumour-secreted substances, and immunological mechanisms have been suggested.Case presentationWe report a 60-year-old Caucasian male attended our hospital with a bulky lymph node mass in the right axilla. Extirpation of a lymph node conglomerate revealed 5 melanoma lymph node metastases. Computed tomography showed a liver metastasis (diameter: 3.8 cm), several retroperitoneal metastases, bilateral metastases in the lung hilus, and prepectoral subcutaneous metastases (Stage IV; pTx, N3, M1c). Lactate dehydrogenase and S100B were slightly elevated. Combination therapy of nivolumab (1 mg/kg BW) and ipilimumab (3 mg/kg BW) was started. Three weeks after the first combination therapy he developed progressive erythema, paraesthesia and pain on the fingertips of both hands. Both cold and warmth was not well tolerated by the patient. Complete work-up excluded associated conditions or factors such as haematological disorders, rheumatologic disorders, hypertension, diabetes or smoking. Treatment was initiated with prostacyclin 20 μg twice daily and oral prednisolone 50 mg in tapering dosage. However, prostacyclin was stopped after the first applications because the pain increased during infusion. The second course of nivolumab and ipilimumab was administered. About 2 weeks later, the patient presented with increased pain and small subungual necrosis. We treated the patient with oral analgetics and intravenous prednisolone 500 mg in tapering dosage. On digital substraction angiography occlusion of all arteries of the fingers was demonstrated. Further rheologic and anti-melanoma treatments were refused by the patient. About 2 months after the second course of nivolumab and ipilimumab combination therapy several fingers showed severe gangrene which finally led to amputations of end phalanges of several fingers. Histopathology did not reveal evidence for vasculitis or other primary vascular pathologies. During the following 2 months the patient experienced dramatic progress of his metastatic disease and finally died at multi-organ failure.ConclusionPresence of rapidly progressive digital ischemia in an elderly patient with cancer should always raise clinical suspicion of a paraneoplastic phenomenon when other possible causes have been excluded. In patients treated with immune checkpoint inhibitors such as CTLA-4 and PD-L1 blockers PVAS-like events have not been reported so far. However, it is debatable whether immune checkpoint blockade may play a pathogenetic role in the development of PAVS in patients with malignancies.

SC13.03 Limitation by COPD for Diagnostic Procedures

Limitation by COPD for Diagnostic Procedures Ostoros, Gy. Varga, J. and Kerpel-Fronius A. National “Korányi” Institute for Pulmonology Hungary, Budapest Lung cancer and COPD are smoking dependent diseases. Smoking cessation is crucial before and during the diagnostic procedures because of the consequences of smoking (e.g. sputum retention, mucociliary dysfunction and potential other more serious complications after the procedures). Severe obstructive or restrictive pulmonary disorders limit the diagnostic possibilities in patients with malignant pulmonary diseases. The screening of lung cancer with low-dose CT has become the gold standard in the past decade. The two imaging-based phenotypes of COPD can be well distinguished by this technique. CT screening can also help to identify non-diagnosed emphysema patients and may lead to early treatment of the disease. It has been showed that non-smoker emphysema patients have a similar risk of lung cancer as smokers with emphysema. Thus, patients with emphysema may be an eligible subgroup for a more intensive lung cancer screening program. However, once a suspicious lesion is found severe COPD, it can limit the choices available for differential diagnosis seriously - CT guided lung biopsies in COPD patients carry a higher risk of hemorrhage and pneumothorax. Patients with severe COPD and respiratory failure with decreased oxygen saturation limit the indication of diagnostic bronchoscopic procedures as well. The examination of exhaled breath condensate (EBC) is a noninvasive process. There are efforts to discriminate lung cancer and COPD with EBC. Lung tumors have influence on lung function. Besides the severity of COPD, the result of the lung function test (LFT) depends on the size and position of the pulmonary tumor as well. In the case of a big central tumor or a huge amount of pleural fluid, the LFT will show rather restrictive than obstructive character. A small peripheral malignancy will not change the shape and volume of the LFT. If the tumor is in the trachea or compresses it's wall, the inspiratory phase of the flow-volume chart could be flat. Sometimes the lung tumors could lead a misdiagnosis of COPD. A centrally located small tumor which is not visible on the chest X-ray but compresses the trachea or any of the pulmonary vessels can cause breathlessness, fatigue and decreased oxygen saturation. A mediastinal conglomerate of lymph nodes can cause similar symptoms. Low physical activity, obesity, smoking and comorbidities are significant negative factors for risk stratification before any pulmonary diagnostic procedure as well. Pulmonary rehabilitation can improve functional reserves if functional capacity is at borderline. Pulmonary rehabilitation has positive effect on cardiovascular function, metabolism, muscle-function and lung mechanics. As for lung function parameters, we need to focus on forced expiratory volume in one second (FEV1) and diffusion capacity (DLCO). We can follow the common agreement of minimum criteria of the European Society of Chest Surgeon and European Respiratory Society for risk stratification before a diagnostic pleuroscopy. Based on this protocol, FEV1 and DLCO need to be >35%pred. In the case of 35%pred< FEV1 and DLCO<75%pred, we need to consider VO2/kg during a cardiopulmonary exercise test. If VO2 is <10 ml/kg/min, the patient need a pulmonary rehabilitation program to improve functional reserves. Regarding lung function, we need to focus on lung mechanics and lung kinematics as well. Lung mechanics can be monitored by resting functional reserve capacity (FRC) and residual volume (RV). Lung kinematics can be monitored by chest expansion. Improved resting or dynamic hyperinflation and lung kinematics of the patients with chest physiotherapy and complex pulmonary rehabilitation is also suggested. As a general effect of rehabilitation, training programs can improve the cardiovascular response, oxygen uptake and the metabolism. We may also focus on physical activity, which is a general prognostics marker. Physical activity can be monitored by pedometer. Obesity can influence the complications of the surgical procedure and it has some effect on lung mechanics as well. If we have time, in case of an obese patient we may also consider improving their body composition before the invasive procedure. To sum up, comorbidities have to be considered before an invasive diagnostic procedure of lung cancer. Patients with impaired pulmonary hemodynamics, ischemic heart disease, diabetes or obesity have to be carefully evaluated. de Torres JP. Casanova C. et al.: Exploring the impact of screening with low-dose CT on lung cancer mortality in mild to moderate COPD patients: A pilot study. Respiratory Medicine, 107, 5, 702-707. 2013. Wiener RS. Schwarcz LM. et al. Population-Based Risk of Complications Following Transthoracic Needle Lung Biopsy of a Pulmonary Nodule. Annals of Internal Medicine, 155, 3, 137-144. 2011. GOLD-www.goldcopd.org Brunelli, A. Charloux, A. et al.: ERS/ESTS clinical guidelines on fitness for radical therapy in lung cancer patients Eur. Resp. J., 34, 1, 2009. diagnosis, copd, lung cancer

Primary Hepatic Neuroendocrine Carcinoma with Multiple Liver Metastases Presenting as Jaundice Due to CBD Obstruction

Background: Whereas more than 80% of NETs found in the liver are metastatic, primary hepatic neuroendocrine tumors (PHNETs) are very rare. We experienced a case of primary hepatic neuroendocrine carcinoma (PHNEC) with multiple liver metastases presenting as jaundice due to the mid CBD obstruction. To the best of our knowledge, few studies have reported similar cases like ours in the English literature. Case presentation: A 81-year-old man was admitted with jaundice of two week's duration. His initial laboratory findings were total bilirubin 19.75 mg/dl, direct bilirubin 15.72 mg/dl, aspartate aminotransferase 65 IU/L, alanine aminotransferase 71 IU/L, alkaline phosphatase 525 U/L, gamma-glutamic transpeptidase 313 U/L and CA19-9 529 U/ml. Abdominal CT revealed marked dilatation of intrahepatic bile duct (IHD) and proximal CBD due to CBD invasion by conglomerated lymphadenopathies in the hepatoduodenal ligament and showed subtly enhancing lesion with central hypodense portion in the liver segment 8/5. MRI with MRCP showed multiple variable sized T2 high signal intensity masses (the largest 3.6 cm) in the both hemiliver, which showed diffusion restriction and delayed enhancement, and diffuse IHD and proximal CBD dilatation with conglomerated lymph nodes at portocaval space and hepatoduodenal ligament. Percutaneous transhepatic biliary drainage was performed, but biopsy could not be done because of concomitant hemobilia. So, we performed sono-guided liver biopsy at the intrahepatic masses. Histopathology revealed poorly differentiated carcinoma. The immunohistochemical staining for CD56, synaptophysin and thyroid transcription factor-1 (TTF-1) revealed positive reaction. So, the diagnosis of neuroendocrine carcinoma, large cell type was confirmed. Chest CT for excluding metastasis from extrahepatic primary site or distant metastatic involvement showed no mass-like lesion in the both lung fields. He refused any methods of treatment such as chemotherapy or transhepatic arterial chemoembolization, and wanted only conservative treatment. Therefore, he was discharged after insertion of self-expandable metal stent at the mid CBD obstruction site and he was died 5 months later. Conclusion: Our case suggests that PHNEC can be presented as multiple intrahepatic masses with CBD obstruction even though PHNETs are very rare. Therefore, it is important to confirm the pathology even in such cases.Figure 1Figure 2

Uropatía obstructiva en cáncer de testículo: características clínicas al momento del diagnóstico oncológico

BackgroundDuring testicular cancer progression, retroperitoneal activity can condition urinary compression, especially in advanced stages. When this presents, it requires urinary diversion. AimTo describe the clinical presentation of obstructive uropathy (OU) associated with testicular cancer at the time of diagnosis, correlating the variables of the neoplasia to establish associations between the 2 entities. Material and methodsA retrospective study encompassing the time frame from July 2010 to June 2015 was conducted that included 98 patients with testicular cancer, evaluating the presence of OU through abdominal tomography and statistical analysis for the purpose of establishing significant associations between the variables. ResultsTwenty patients (20.2%) presented with OU, with a mean age of 24 years, ECOG 1, and a progression time of 305 days. Eight patients had seminomatous tumor (40%) and 12 had nonseminomatous tumor (60%), of which the most frequent histology was endodermal sinuses (66%), followed by embryonal carcinoma and teratoma (50%, respectively). The mean size of the lymph node conglomerate was 4.3cm, 65% of the patients had metastasis upon diagnosis, and the most frequent clinical stage was IIIC (50%). Forty percent of the patients had elevated creatinine.There were no statistically significant results between the development of retroperitoneal disease and histology (P=0.72), progression time (P=0.44), or laterality (P=0.44).Testicular tumor size was correlated with the development of OU (P<0.05), the same as was stage pT (P<0.05), stage N (P<0.05), metastases (P=0.001), LDH (P<0.05), clinical stage (P<0.05), and young age (P<0.05) ConclusionsOU is a comorbidity associated with germ cell testicular cancer that usually involves advanced stages. Young patients with poor functional status, with large tumors, and elevated LDH levels after orchiectomy should be evaluated to rule out urinary compression.

Tuberculous liver abscess in an immunocompetent child with pulmonary tuberculosis as a cause of fever of unknown origin

Infectious diseases are the leading cause of FUO. A case of prolonged fever with hepatic and pulmonary tuberculosis as a final diagnosis is herein presented. A 4-year-old, otherwise healthy boy presented with an axillary temperature of up to 39.5&amp;#xba;C for the previous 3 weeks. His medical history revealed an occasional increase in body temperature up to 38.5&amp;#xba;C for the last 6 months. Physical examination revealed coarse breath sounds on the basal lung area. Chest X-ray showed mediastinal lymphadenomegaly and computed tomography revealed paratracheal conglomerated lymph nodes and a groundglass appearance on the right lung. There were multiple contrast-enhanced, hypoechoic nodules with central necrosis in the liver parenchyma on abdominal magnetic resonance imaging. Open liver biopsy yielded chronic granulomatous inflammation compatible with pathological findings of tuberculosis infection. The culture specimen was positive for Mycobacterium tuberculosis. The patient improved rapidly after antituberculous therapy was initiated. Tuberculosis, especially in its disseminated form, poses a distinct diagnostic challenge in cases of prolonged fever with unproven etiology, and thus persistence should be exercised in disclosing the cause of such fevers.

Influence of histopathologic factors on pattern of metastasis in squamous cell carcinoma of the head and neck

Knowledge of histopathological factors, such as perineural invasion (PNI), extracapsular spread (ECS), angiolymphatic vessel invasion, and conglomerate lymph nodes, in patients with head and neck squamous cell carcinoma is important for appropriate treatment decisions. Given the availability of aggressive therapeutic options with known side effects and burdens for the patient, choosing the correct treatment option is vital. Retrospective patient database. Using a retrospective database of patients treated over a 10-year period, independent prognostic factors for disease-free survival and overall survival were assessed. Univariate analysis was used to identify significant variables, and multivariate Cox regression analysis was used to determine independent prognostic factors. Between January 1, 1999 and December 31, 2009, 291 patients with head and neck squamous cell carcinoma were analyzed to identify influence of histopathological factors on pattern of metastasis. PNI tends to set metastasis locoregional, both in ipsilateral and contralateral lymph nodes. Lymphangiosis eases the way toward ipsilateral lymph node metastasis. ECS spread and hemangiosis demonstrated a significantly higher risk of distant metastasis. With the occurrence of conglomerate lymph nodes, patient data demonstrated a highly significant accumulation of distant metastasis. Patients with PNI and lymph vessel invasion tend toward locoregional lymph node metastasis on the ipsilateral side of the neck. In cases of PNI, the patient is at risk for a contralateral neck recurrence of the tumor. In patients with ECS, blood vessel invasion, or conglomerate disease, disseminated spread of the disease to distant locations is more likely and has a high negative impact on survival of the patient.

Splenic Diffuse Red Pulp Small B Cell Lymphoma: Transformation To Diffuse Large Cells B Lymphoma

ObjectiveReport immunophenotypic and genetic aspects and clinical course of a case of Splenic Diffuse Red Pulp Small B Cell Lymphoma with transformation to diffuse large cell. IntroductionUntil recently, only Hairy Cell Leukemia was recognized as B chronic lymphoproliferation with primary involvement of the splenic red pulp. The 2008 WHO classification included two new provisional entities: Splenic diffuse red pulp small-B cell lymphoma and Hairy Cell Leukemia variant. These entities are rare, encompassing less than 1% of B chronic lymphoproliferation, characteristically presenting as an indolent clinical course and good control with splenectomy. Case reportFemale, 29 years old, with non-replicative chronic hepatitis B, and splenomegaly detected 7 years before without evidence of portal hypertension or schistosomiasis. Two months before admission to our hospital, she developed increasing splenomegaly for 39cm, B symptoms and abdominal lymphadenopathy. CBC showed Hb: 8,1g/dl, platelet: 80.000/mm3, WBC: 12.000/mm3 with 85% of abnormal lymphoid cells with immunophenotyping CD45++/CD19+/CD20++/CD25+/CD23dim/CD200dim/FMC7+/CD11c-/CD103- and cykappa restriction. Complex karyotype in PB suggested lymphoproliferative disorder, with strutural abnormalities in 14q32, isocromosomes 8 and 17, deletions 6q and 7q and trisomy 18. FISH studies showed extra copy of MYC and p53 deletion. Bone marrow aspirate showed 7,6% of cells with same phenotype. CT and PET-CT showed splenomegaly of 39cm (SUV: 6.1), hepatomegaly (SUV: 3.2), abdominal lymph node conglomerate (SUV: 6.3), cervical and mediastinal lymphadenopathy with no increase in dimensions (SUV: 3,8). The patient was submitted to splenectomy and histological findings revelead mature lymphoid neoplasm, ranging from small to intermediate cells with few large cells, primary from spleen red pulp and diffusely infiltrating the sinusoidal spaces and secondly the white pulp (CD20+, DBA44+, CD5-, CD10-, CD23-, CD3-, cyclin D1- and Ki67: 40%). The cytogenetic analysis of the spleen showed similar complex karyotype as seen in PB. With the diagnosis of Splenic Diffuse Red Pulp Small B Cell Lymphoma with aggressive transformation to diffuse large cell, she was submitted to 8 cycles of R-CHOEP and is in complete remission, waiting for consolidation with high-dose chemotheraphy followed with peripheral stem cells rescue. DiscussionSplenic Diffuse Red Pulp Small B-cell Lymphoma was included as a provisional entity in WHO 2008 classification. The few cases reported show an indolent course with good response to splenectomy. In this case the diagnosis was suspect with clinical course, cytogenetic and flow cytometry PB studies and confirmed after splenectomy when the clinical course of disease changed with B symptoms, spread to peripheral blood with atypical cells and increased proliferative markers, such as LDH. This fact highlights the difficulty in establishing the diagnosis of this entity during the course of prolonged and indolent disease, manifested by splenomegaly oligosymptomatic often neglected by physicians and by the patients themselves. Disclosures:No relevant conflicts of interest to declare.

A Case of Diffuse Large B Cell Lymphoma of Stomach Presenting Like a Volcano Ulcer in Klinefelterʼs Syndrome Patient

Purpose: Klinefelter's syndrome, the most common congenital abnormality causing primary hypogonadism, is associated with breast cancer, germcell tumours, and nonhodgkin lymphomas. A 53-year-old male came with fatigue, poor appetite, weakness, 51 pounds of weight loss, and night sweats in the last year. He started having nausea, vomiting with specks of blood and sharp abdominal pain mainly in epigastric region. He denied any medication use. He used to smoke. Family history was noncontributory. On admission, his vitals were stable. Epigastric tenderness was present, otherwise his examination was benign. His hemoglobin was 7.3 with MCV of 73.6, albumin was 1.9 and INR was 1.43. HIV test was negative. LDH was 152, hep B and hep C tests were negative. CT chest, abdomen and pelvis with contrast revealed splenomegaly, mesenteric and retroperitoneal lymphadenopathy. Endoscopy showed deep ulcer on the lesser curvature of the stomach approximately 7 cm from the GE junction with irregular friable borders and volcano appearance, and acute gastritis was found in the antrum. Biopsy showed diffuse large B cell lymphoma. Bone marrow biopsy was negative for lymphoma. Cytogenetic analysis on bone marrow showed XXY (Klinefelter syndrome). PETCT revealed diffuse irregular thickening with intense uptake in the proximal stomach consistent with gastric lymphoma. Multiple conglomerate lymph nodes with intense uptake in the gastrohepatic ligament, gastrosplenic space, preaortic, aortocaval, peripancreatic, celiac, mesenteric and left common iliac regions, the largest measuring about 6 cm, in the left periaortic region and abutting the pancreatic body and tail. All were consistent with lymphoma. Mild splenomegaly was present without abnormal uptake. Stage IIIB DLBCL (diffuse large B cell lymphoma) was diagnosed and the patient was started on treatment with 6 cycles of R-CHOP. Repeat PETCT showed incredible response of the gastric and nodal lymphomatous lesions to chemotherapy compared to the previous one. Repeat endoscopy showed complete resolution of previous lesions. Klinefelter's syndrome patients may be at substantially elevated risks for non-Hodgkin's lymphoma. Studies have shown lymphocytes in Klinefelter's syndrome patients have higher sensitivity than normal control cells when exposed to radiation or mitomycin C. Cells of Klinefelter's syndrome were 3-10 times more easily transformable than normal cells. Review of the literature suggested men with Klinefelter's syndrome have increased incidence of non-Hodgkin's lymphomas. They suggested a possible combination of immunological deficency, endocrinological factors and inability to control hematopoiesis as an etiological factor.

Don't Blame It on the Scar Tissue

Question: A 61-year-old white woman was evaluated for complaints of intermittent attacks of a sharp, severe, periumbilical abdominal pain for 15 years with recent worsening in severity and nocturnal episodes. Her symptoms were partially alleviated by bowel movements and no worsening factors were identified. Her past medical history was significant for a total hysterectomy and adhesiolysis. Physical examination revealed prior surgical incisions over the abdominal wall and otherwise benign on examination. She denied fever, nausea, vomiting, painful adenopathy, weight loss, night sweats, recent infectious exposures, or foreign travels. Laboratory studies, including a complete blood cell count with white blood cell differential, renal profile, hepatic transaminases, erythrocyte sedimentation rate, serum electrophoresis, and 5-hydroxyindoleacetic acid levels, were negative or within normal values. Esophagogastroduodenoscopy and colonoscopy were unremarkable. A tuberculin purified protein derivative test was negative as was computed tomography (CT) of the chest. CT enterography (Figure A) showed enlarged mesenteric lymph nodes in the right mid abdomen (arrows). Capsule endoscopy and upper and lower double balloon enteroscopy with complete visualization of the entire small bowel lumen were unremarkable. Because of persistent symptoms, follow-up CT enterography was performed 8 weeks later and showed interval increase in the size of the conglomerate lymph nodes masses in the right side of the mesenteric fat, as well as cicatricial changes involving loops of bowel adjacent to the mesenteric masses. CT-guided fine-needle aspiration biopsy was performed. What is the diagnosis? Look on page 914 for the answer and see the Gastroenterology web site () for more information on submitting your favorite image to Clinical Challenges and Images in GI. Fine-needle aspiration showed fat necrosis (arrow) with surrounding fibroblastic proliferation, dense fibrosis and admixed inflammatory infiltrate (Figure B, medium power). A high power image (Figure C) demonstrated inflammatory cells consisting of mononuclear leukocytes, histiocytes, and scattered plasma cells with background of bland fibroblasts and fibrosis. There was no evidence of malignant cells, lymphoma, or carcinoma. These findings were most consistent with a diagnosis of sclerosing mesenteritis (SM). First described in the 1920s, SM is a rare, idiopathic, nonmalignant inflammatory disease that generally affects the small bowel mesentery.1Akram S. Pardi D.S. Schaffner J.A. et al.Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients.Clin Gastroenterol Hepatol. 2007; 5: 589-596Abstract Full Text Full Text PDF PubMed Scopus (210) Google Scholar The etiology is unknown and has been linked with a medical history of abdominal surgeries, trauma, paraneoplastic syndrome, and autoimmunity.2Emory T.S. Monihan J.M. Carr N.J. et al.Sclerosing mesenteritis, mesenteric panniculitis and mesenteric lipodystrophy: a single entity?.Am J Surg Pathol. 1997; 21: 392-398Crossref PubMed Scopus (274) Google Scholar SM is part of a spectrum of an inflammatory process of the mesentery that may manifest at different stages, including fat necrosis (lipodystrophy), chronic tissue inflammation (mesenteric panniculitis), and fibrosis (SM).2Emory T.S. Monihan J.M. Carr N.J. et al.Sclerosing mesenteritis, mesenteric panniculitis and mesenteric lipodystrophy: a single entity?.Am J Surg Pathol. 1997; 21: 392-398Crossref PubMed Scopus (274) Google Scholar Affecting men more than women (2:1) in the fifth to seventh decades of life, its nonspecific presenting symptoms represent a diagnostic challenge for physicians.3Avelino-Silva V.I. Leal F.E. Coelho-Netto C. et al.Sclerosing mesenteritis as an unusual cause of fever of unknown origin: a case report and review.Clinics (Sao Paulo). 2012; 67: 293-295Crossref PubMed Scopus (7) Google Scholar Diagnosing SM includes the use of laboratory and imaging tests, with CT of the abdomen and pelvis being the most sensitive and useful, showing a soft tissue mass in the small bowel mesentery. However, the gold standard is biopsy.2Emory T.S. Monihan J.M. Carr N.J. et al.Sclerosing mesenteritis, mesenteric panniculitis and mesenteric lipodystrophy: a single entity?.Am J Surg Pathol. 1997; 21: 392-398Crossref PubMed Scopus (274) Google Scholar When concerning signs are present (eg, weight loss, anemia or night sweats), further workup is recommended to rule out a lymphoproliferative disorder.1Akram S. Pardi D.S. Schaffner J.A. et al.Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients.Clin Gastroenterol Hepatol. 2007; 5: 589-596Abstract Full Text Full Text PDF PubMed Scopus (210) Google Scholar Thirty percent of patients may present with intra-abdominal malignancies, the majority being lymphoma.1Akram S. Pardi D.S. Schaffner J.A. et al.Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients.Clin Gastroenterol Hepatol. 2007; 5: 589-596Abstract Full Text Full Text PDF PubMed Scopus (210) Google Scholar Treatment is offered to symptomatic patients. A regimen of tamoxifen and glucocorticoids has been described, as well as use of other anti-inflammatory, immunomodulatory, and antifibrotic agents; bowel obstruction is treated with surgery.1Akram S. Pardi D.S. Schaffner J.A. et al.Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients.Clin Gastroenterol Hepatol. 2007; 5: 589-596Abstract Full Text Full Text PDF PubMed Scopus (210) Google Scholar The patient was placed on a prednisone taper and started on tamoxifen 10 mg twice daily. Within 3 weeks, the intermittent abdominal pain attacks resolved completely and she continues to remain asymptomatic.

A chronic ulcerative wound in the neck and disseminated tuberculosis: a case report and review of the literature

A 79-year-old woman was admitted to the emergency room with an ulcerated large wound in the lateral region of her neck. Computed tomography (CT) of the thorax revealed air bronchograms, nodular infiltration and areas of consolidation areas in both lungs. Multiple conglomerated lymph nodes, tending to coalesce with each other with areas of necrosis, were observed on the neck CT. Numerous acid-fast bacilli were seen on wound microscopy and in sputum. A case of disseminated tuberculosis is presented to draw attention to the dangers of delayed diagnosis despite improved tuberculosis treatment regimens.

Fibrosing mediastinitis mimicking bronchogenic carcinoma.

Fibrosing mediastinitis is a rare but benign disorder characterized by an excessive fibrotic reaction in the mediastinum which can result in compromise of airways, great vessels, and other mediastinal structures. In this paper we presented a patient with fibrosing mediastinitis mimicking bronchogenic carcinoma. The patient was a 32-year-old diabetic male admitting with cough and hemoptysis. There was a right hilar mass and multiple mediastinal conglomerated lymph nodes on chest computed tomography. Positron emission tomography with computed tomography (PET/CT) scan demonstrated increased fluorodeoxyglucose (FDG) uptake at the right hilar mass lesion and mediastinal lymph nodes. Fiberoptic bronchoscopy showed mucosal distortion of right upper lobe. Pathologic examination of the mucosal biopsy revealed inflammation. Endobronchial ultrasound guided transbronchial needle and cervical mediastinoscopic lymph node biopsies were undiagnostic. Diagnostic thoracotomy confirmed the diagnosis fibrosing mediastinitis. Administration of six months of systemic corticosteroid and antituberculous therapy was not beneficial. In conclusion, despite being a rare clinical entity, fibrosing mediastinitis should be kept in mind in the differential diagnosis of mediastinal mass lesions of unknown etiology. The diagnosis is exceptionally difficult in the presence of atypical radiological findings. The treatment is particularly challenging without any proven effective therapy.

Automatic Detection and Segmentation of Lymph Nodes From CT Data

Lymph nodes are assessed routinely in clinical practice and their size is followed throughout radiation or chemotherapy to monitor the effectiveness of cancer treatment. This paper presents a robust learning-based method for automatic detection and segmentation of solid lymph nodes from CT data, with the following contributions. First, it presents a learning based approach to solid lymph node detection that relies on marginal space learning to achieve great speedup with virtually no loss in accuracy. Second, it presents a computationally efficient segmentation method for solid lymph nodes (LN). Third, it introduces two new sets of features that are effective for LN detection, one that self-aligns to high gradients and another set obtained from the segmentation result. The method is evaluated for axillary LN detection on 131 volumes containing 371 LN, yielding a 83.0% detection rate with 1.0 false positive per volume. It is further evaluated for pelvic and abdominal LN detection on 54 volumes containing 569 LN, yielding a 80.0% detection rate with 3.2 false positives per volume. The running time is 5-20 s per volume for axillary areas and 15-40 s for pelvic. An added benefit of the method is the capability to detect and segment conglomerated lymph nodes.

Radioguided lymph node biopsy of a chemoresistant lymph node detected on interim FDG PET-CT in Hodgkin lymphoma

A 32-year-old male patient was diagnosed with nodular lymphocyte-predominant Hodgkin lymphoma. Staging FDG PET-CT detected a large right axillary lymph node conglomerate and splenic manifestation. Interim PET-CT following two cycles of ABVD chemotherapy revealed good metabolic response with the exception of a single axillary lymph node. A second "interim" PET-CT after two further cycles showed a similar result. A biopsy of the metabolically active non-palpable lymph node was performed using radioguided occult lesion localization (ROLL) with ultrasound guidance. The lymph node was successfully removed by minimally invasive surgery. Histological evaluation of the lymph node revealed a T cell-rich diffuse large B cell lymphoma. Based on this finding, a more aggressive treatment regimen followed by high dose chemotherapy with autologous stem cell rescue was adopted. To our knowledge, this is the first report of a lymphoma case in which the ROLL method was used in the radioguided biopsy of a chemoresistant lymph node detected by interim FDG PET-CT.

Linfoadenitis criptococócica mesentérica: Una rara causa de abdomen agudo. Caso clínico

Cryptococcosis is an invasive mycotic infection caused by Cryptococcus neoformans, an encapsulated, yeast-like fungus. It is considered an opportunist infection, since it mainly affects immunocompromised subjects. However there are isolated reports of the infection in immunocompetent subjects. Cryptococcal infection of intra-abdominal organs or tissues is extremely rare. We report a 21-year-old HIV positive male that, during the treatment of a meningeal cryptococcosis, presented a clinical picture of an acute abdomen suggesting acute appendicitis. The patient was operated, finding enlarged mesenteric lymph nodes forming conglomerates and a macroscopically normal appendix. The conglomerated lymph nodes and the appendix were excised. The pathological study of the surgical piece revealed an intra abdominal cryptococcal lymphadenitis and a normal appendix.

A Case of Advanced Medullary Thyroid Carcinoma Successfully Treated with Sunitinib

Medullary thyroid carcinoma (MTC) is a neuroendocrine tumor arising from "C" cells of the thyroid; it is a RET associated cancer that can be sporadic or familial in origin. Advances in understanding the genetic changes associated with the development of MTC explain the growing interest in the therapeutic potential of tyrosine kinase inhibitors. Sunitinib is an orally administered multikinase inhibitor likely to target multiple pathways in the tumor, stromal, and endothelial compartments. Its role in the treatment of MTC patients has not yet been established. To present the case of a patient with a sporadic and unresectable MTC who was successfully treated with sunitinib. A 55-year-old man with locally advanced MTC, without germinal and/or somatic RET mutations, was started on sunitinib (50 mg/day for 28 days, followed by 14 days of no treatment). At the time of writing, he had received four consecutive cycles. At the end of the first cycle, his serum calcitonin level had dropped by 81%. In the following cycles, a long-lasting minor response was observed. An early and dramatic tumor reduction, particularly of a cervical lymph node conglomerate, was observed and confirmed by the Response Evaluation Criteria in Solid Tumors. Sunitinib may play a role in the management of patients with locally advanced MTC or distant metastatic disease, for which no effective systemic therapy exists. Moreover, the absence of RET mutations does not seem to be an exclusion criterion for sunitinib treatment.

Lymphknotensarkoidose der Submandibularisloge

Sarcoidosis is a granulomatous systemic disease of unknown etiology. Besides the landmark pulmonary lesions, extrathoracic manifestations of the disease can also occur. We report the case of a 53-year-old woman with an obscure swelling of both submandibular compartments. The radiological and pathohistological evaluations confirmed the uncommon diagnosis of sarcoidosis of the submandibular compartment. The tumor in each compartment consisted of a huge lymph node conglomerate respectively displacing the submandibular gland. The major salivary glands and the thorax were not involved.

Multilocular cervical thymic cyst: an unusual neck mass in children

Thymic cysts are usually considered uncommon lesions in the differential diagnosis of neck masses. Approximately 100 cases have been reported to date and most of these cases have occurred asymptomatically. Herein, the authors report a 4-year-old boy with cervical thymic cyst, which was misdiagnosed preoperatively as conglomerated lymph nodes. The correct diagnosis was made after surgical excision and through determination of specific histopathological findings of the thymic cyst. The etiologic theories, differential diagnosis and the treatment of thymic cysts are discussed.

Percutaneous retroperitoneal lymph node biopsy: An appraisal for a substitute to laparotomy in far advanced metastatic carcinoma

In order to establish the true extent of disease and more accurately prescribe extended field radiotherapy and/or chemotherapy, percutaneous needle biopsy techniques have been employed. Although there is a relatively high correlation between lymphangiographic appearances and the spread of tumor in patients with advanced disease, this has not been the experience in patients with clinically staged early disease. Unfortunately, a metastases is not the only cause of a filling defect in a lymph node. Similar defects may be caused by fibrosis or fatty replacement, hilar structures, or conglomerate lymph nodes. Using a 23-gauge, 15-to 20-cm needle with local anesthesia and biplane flouroscopy, 50 patients underwent a percutaneous needle biopsy. In 33% of the patients, the staging of disease and management was enhanced by using this technique. The percutaneous needle biopsy technique was compared for accuracy of diagnosis with lymphangiography and surgery. The overall accuracy with the percutaneous needle biopsy technique was 86% as opposed to the lymphangiogram 38% and surgery 66%. This technique appears to be a relatively safe procedure for evaluation of the true extent of disease and enhancing therapy.