Full dose neoadjuvant FOLFIRINOX is associated with prolonged survival in patients with locally advanced pancreatic adenocarcinoma.

BACKGROUND: The efficacy of FOLFIRINOX for metastatic pancreatic cancer has led to its use in patients with earlier stages of disease. This study retrospectively analyzed a cohort of patients with locally-advanced pancreatic cancer (LAPC) treated with FOLFIRINOX. METHODS: Between 2008 and 2013, 51 treatment-naïve patients with LAPC at a single institution received first-line FOLFIRINOX with neoadjuvant intent, at the full dose as described in the PRODIGE 4/ACCORD 11 study. Combined chemoradiation was administered for those who remained unresectable after maximum response to chemotherapy. The primary outcome measure was overall survival (OS), and secondary outcomes were progression-free survival (PFS) and margin-negative (R0) resection rate, and toxicity profile. RESULTS: A total of 429 cycles of FOLFIRINOX were given with a median of 8 cycles (range 2-29) per patient; 66% of cycles were full dose. After chemotherapy, 27 (53%) received chemoradiation. The median OS was 35.4 months (95% CI 25.8-45). Ten (4 borderline resectable and 6 unresectable) patients had successful R0 resections; those who had R0 resections had a significantly longer survival than those who did not (3-year OS rate 67% versus 21%, log rank p = 0.042). Increasing number of full-dose cycles was significantly associated with increased survival. The toxicity profile was similar to previous reports of this regimen. CONCLUSIONS: FOLFIRINOX is feasible as neoadjuvant therapy for LAPC. Although the R0 resection rate was only 20%, the median OS of almost 3 years appears promising. Dose intensity and duration were associated with increased survival in this study, arguing against dose attenuated versions of this regimen.

Association between chronic hepatitis C and hepatitis C/HIV co-infection and the development of colorectal adenomas.

BACKGROUND: Limited knowledge exists about the effects chronic hepatitis C virus (HCV) infection has in the development of colorectal adenomas (CRA). Data regarding the association between chronic HIV infection and the development of CRA is scarce as well. We aim to determine if there is an association between the development of CRA and chronic infection with HCV and HCV/HIV co-infection. METHODS: From July 1, 2009 to March 31, 2011 a total of 2,051 patients that underwent colonoscopy were included in our study. The population was divided into 2 study groups: those patients who tested positive for HCV, and HCV/HIC; the control groups consisted of patients whose results were negative. Fisher's exact χ(2) test for categorical variables and t-test for continuous variables was used to analyze data between groups. Logistic regression was performed to obtain odds ratios (OR). RESULTS: CRA detection was higher in the HCV than in the control group (26.3% vs. 20.2%; P=1.02); Likewise, the incidence of CRA (25.5% vs. 20.8%; P=0.63) was higher in the co-infection group. However, in both of the study groups this difference was non-statistical. CONCLUSIONS: A higher detection rate of CRP was seen in the HCV population; however, it failed to reach statistical significance. Whether co-infection with HIV/HCV increases the incidence of CRA and/or has a synergistic effect remains to be determined. The small sample population and the retrospective single institution nature of our study, as well as other confounders may have contributed to our negative results. However, our findings question whether HCV and HIV/HCV co-infected patients will benefit from screening colonoscopy at an earlier age. This issue merits further investigation with a large multi-center prospective study.

Synchronous Small Cell Neuroendocrine Carcinoma and Adenocarcinoma of the Colon: A Link for Common Stem Cell Origin?

Synchronous carcinomas have been recognized for over a century, with synchronous primary adenocarcinoma of the colon reported to range from 2-11% of cases involving this type of malignancy. Small cell carcinomas occur frequently with colorectal adenomas; however, despite these reports and a known adenoma-to-carcinoma sequence, scarce literature exists on synchronous colorectal adenocarcinoma and small cell carcinomas. We present a rare cancer of synchronous small cell neuroendocrine carcinomas and discuss a possible link between these two cancers.

Budd-Chiari syndrome induced by stage IV rectal carcinoid.

Budd-Chiari syndrome (BCS) is defined as an obstruction of the hepatic venous outflow anywhere from the small hepatic veins to the suprahepatic inferior vena cava. In this study, a rare case of BCS induced by a metastatic rectal carcinoid is presented. A 57-year-old African American woman with stage IV rectal carcinoid presented with right upper quadrant pain, associated with decreased appetite and weight loss >13 kg over 2 months. Computed tomography scan with contrast enhancement revealed filling defects in the left and middle hepatic veins extending into the suprahepatic inferior vena cava to the junction of the right atrium, suggesting BCS. Thrombophilia workup was negative, and no signs of liver cirrhosis or portal hypertension were found. A hepatitis profile workup yielded negative results. This is the first reported case of BCS that is associated with a metastatic rectal carcinoid. More research is needed to identify the mechanism leading to thrombogenesis in carcinoid tumors.

Obesity, metabolic factors, and colorectal adenomas: a retrospective study in a racially diverse New York State Hospital.

OBJECTIVE: We studied a racially diverse population and the relationship with colorectal adenomas (CA) further looking for risks related to BMI and metabolic factors. DESIGNS: Seven hundred seventy-nine patients who underwent screening colonoscopies between 2007 and 2009 meeting exclusion criteria were included. To evaluate the association between race, BMI, and other metabolic factors with having one or more CA detected at colonoscopy, adjusted odds ratios and 95 % CI were estimated using unconditional logistic regression models. OUTCOMES: CA were detected in 167 out of 779 (21.4 %) patients. Compared to Whites, Hispanics were less likely to have one or more adenomas detected during a screening colonoscopy (OR = 0.52, 95 % CI, 0.31-0.88; p = 0.01). There was no significant statistical difference between Blacks and Whites, or other races and Whites. There was an association between the presence of CA and smoking (OR = 1.57, 95 % CI, 1.02-2.43; p = 0.04). CONCLUSION: Our results showed that Hispanics were less likely to have an adenoma detected during a screening colonoscopy than Whites. No statistical significant difference was found between patients with metabolic factors and the presence of colorectal adenoma.

Inflammatory malignant fibrous histiocytoma associated with leukemoid reaction or leukocytosis: a comprehensive review.

Inflammatory malignant fibrous histiocytoma (IMFH) associated with leukemoid reaction (LR)/leukocytosis is a rare entity. In this paper, we search PubMed for all known cases of IMFH associated with LR/leukocytosis in an attempt to draw conclusions about this variant's response to treatments and its pathophysiology. Medline electronic database was searched using key words such as malignant fibrous histiocytoma, leukemoid reaction, and leukocytosis. A total of 16 patients were found, twelve males (75%) and 4 female (25%), with a mean age of 62.6 years, ranging from 47 to 77. The mean survival was 770 days, ranging from 14 to 6570 days. Four patients were alive at last follow-up: 6570 days, 1095 days, 335 days, and 180 days, respectively. Of the 12 patients that expired, death occurred approximately 92 days after the onset of LR or leukocytosis, ranging from 3 to 334 days. We conclude that IMFH associated with LR/leukocytosis does not completely respond to chemoradiation. Overproduction of growth factors and cytokines by IMFH cells and their interactions with the inflammatory infiltrate seem to promote immunological effector cell's dysfunction and substantiate the development and growth of this neoplasm. A clear understanding of these molecular pathways is crucial in order to identify targets for potential therapy.

Cutaneous inflammatory malignant fibrous histiocytoma presenting with a leukemoid reaction: a case report and review of the literature.

Malignant fibrous histiocytoma (MFH) is the most common sarcoma found in adults. We discuss a case of inflammatory MFH of dermal/epidermal origin presenting with a severe leukemoid reaction (LR). A 60 years old white male presented to hematology/oncology clinic complaining of mild shortness of breath on exertion. Past medical history was remarkable for removal of a left upper extremity necrotic mass 4.4 × 3 × 3 cm. Microscopy of the specimen showed clear surgical margin, and tumor cells restricted to the dermis without lymphovascular invasion. Immunohistochemestry was positive for CD 68 and CD 99. Chest x-ray was negative for metastatic disease. White blood cell count was 109.4 k/mm(3) with 24 k/mm(3) band neutrophils, and absolute neutrophil count of 69 k/mm(3). CT scan of the thorax revealed numerous bilateral pulmonary nodules suspicious for metastasis. Based on these findings patient was diagnosed with metastatic cutaneous IMFH associated with a LR. Following review of medical literature, this appears to be the first reported case of inflammatory cutaneous MFH associated with LR. This histological variant is rare, and carries a poor prognosis. Thus, we would like to emphasize the need for investigating alternative therapies capable of improving the survival of these patients.

Synchronous Duodenal Carcinoid and Adenocarcinoma of the Colon.

Carcinoid tumors are a histological subtype of well differentiated, low to intermediate grade, slow-growing neuroendocrine malignancies capable of secreting bioactive peptides, such as 5-hydroxytryptamine (5-HT, serotonin), chromogranin-A and chromogranin-C. Here we present a case of a duodenal carcinoid that simultaneously occurred with adenocarcinoma of the colon. A 59-year-old male with a past medical history of hepatitis C and hypertension presented complaining of worsening abdominal pain associated with 2 - 3 episodes per week of bright red blood per rectum for the past month. He also reported a 20 pounds weight loss in the last 6 months. Social history was significant for a 15 pack year history. Vitals on admission were within normal limits. Physical exam was significant for right upper quadrant tenderness without guarding, rebound, or organomegaly. Rectal exam revealed no blood or masses. Laboratory results showed iron deficiency anemia with hemoglobin of 9.6 K/mm3. Esophagogastroduodenoscopy revealed a 4 mm duodenal polyp. Colonoscopy was terminated early secondary to a large circumferential obstructing mass found in the descending colon. Immunohistochemistry of the duodenal biopsy was positive for synaptophysin and chromogranin-A; consistent with the diagnosis of stage I carcinoid tumor. Biopsy results of the colonic mass showed a stage I well-differentiated adenocarcinoma. The patient underwent a left colectomy and partial duodenectomy; he remains in remission after 2 year of close follow up. When the diagnosis of small bowel carcinoid is made, further screening for other primary neoplasms should be sought to prevent potential late stage diagnosis of synchronous malignancies. This is crucial because patients' demise usually result from the associate tumor and not the carcinoid component. Finally, we would like to raise clinician's awareness regarding the incidence of this entity since some of the studies suggest that it is more common than it was previously thought.

Lymphocyte Rich Hodgkin's Lymphoma Presented with Warm Hemolytic Anemia: A Case Report and Literature Review.

Hodgkin's lymphoma accounts for ten percent of all lymphomas. In the United States, there are about 8000 new cases every year. This paper describes a case of lymphocyte-rich Hodgkin's lymphoma (LRHL) manifested by autoimmune hemolytic anemia (AIHA). A 27-year-old Israeli male presented with dizziness associated with one month of low-grade fevers and night sweats; he also complained of persistent cough, pruritus, and ten-pound weight lost during this time. The CBC revealed hemoglobin of 5.9 gm/dL, and direct Coomb's test detected multiple nonspecific antibodies consistent with the diagnosis of AIHA. Chest, abdomen, and pelvic CT scan showed mediastinal lymphadenopathy and splenomegaly. Lymph node biopsy revealed classic LRHL. AIHA resolved after completion of the first cycle of chemotherapy with adriamycin, bleomycin, vinblastine, and dacarbazine (ABVD); after six cycles, he went into complete remission. Although infrequent, AIHA can be responsible for the presenting symptoms of HL.

Diagnostic challenge of hepatopulmonary syndrome in a patient with coexisting structural heart disease.

Hepatopulmonary syndrome (HPS) is a severe complication seen in advance liver disease. Its prevalence among cirrhotic patients varies from 4-47 percent. HPS exact pathogenesis remains unknown. Patient presents with signs/symptoms of chronic liver disease, and dypsnea of variable severity. Our patient is a 62 years old white male with a known history of chronic hepatitis C, cirrhosis, ascites, and hypothyroidism who presented to GI/liver clinic complaining of 1 episode BRBPR, and exacerbating dypsnea associated with nausea and few episodes of non-bloody vomit. Physical exam showed, icterus, jaundice, few small spider angiomas on the chest, decrease breath sounds bilateral right more than left, and mild tachycardic. Abdominal exam revealed mid-line scar, moderated size ventral hernia, distention, diffused tenderness, and dullness to percussion. Laboratory result: CBC 5.2/13.2/37.6/83, LFTs 83/217/125/5.2/4.7/7.4, Pt 22.6 INR 1.9 PTT35.4. CT scan showed liver cirrhosis, abdominal varices, and moderated ascites collection around ventral hernia. Calculated A-a gradient was 49.5. Echocardiography revealed patent foramen ovale (PFO) with predominant left to right shunt. In our case, existence of paten foramen ovale (PFO) and atelectasis precludes definitive diagnosis of HPS. Presence of cardiopulmonary shunt could be partially responsible for the patient's dypsnea exacerbation.

A Case of an Unusually Aggressive Cutaneous Anaplastic Large T-Cell Lymphoma in an HIV Patient Treated with CHOP.

Anaplastic large cell lymphoma (ALCL) is the second most common malignancy of T-cell phenotype. This case report describes an unusual rapidly progressing cutaneous anaplastic large T-cell lymphoma in an HIV patient. Our patient is a twenty-year-old African American male with perinatally acquired HIV who presented with a 2 × 2 centimeter necrotic lesion in the right 1st toe; however, 2-3 weeks later multiple smaller lesions appeared on the anterior aspect of the right foot, ankle, and thigh. Biopsy showed cells strongly positive for CD3 and CD30 and negative for CD56 and the ALK gene product. CT of the chest, abdomen, and pelvis was negative for extracutaneous involvement favoring cutaneous ALCL. Patient was treated with 6 cycles of CHOP (cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisone) chemotherapy and went into complete remission. Due to the aggressive course that this malignancy follows in HIV patients we suggest prompt treatment with systemic therapy.