Boris Avezbakiyev

Hepatocellular Carcinoma Presenting as an Incidental Isolated Malignant Portal Vein Thrombosis

IntroductionPortal vein thrombosis is frequently associated with hepatocellular carcinoma (HCC). Tumor invasion into the portal vein by direct venous extension or metastasis occurs in up to 70% of HCC patients (Cedrone et al., Liver 16:94–8, 1996). However, presentation as an isolated malignant portal vein thrombosis without any evidence of obvious hepatoma-like lesions in the liver by imaging studies is extremely uncommon. We present an unusual case of HCC presenting as a malignant portal vein thrombus, proven on biopsy of the thrombus without any evidence of primary liver lesion. This, to our knowledge, is the first case of HCC presenting as an incidental isolated malignant portal vein thrombosis. The importance of doing delayed enhancement imaging studies to rule out malignant portal vein thrombosis is emphasized.Case ReportA 60-year-old man presented with acute substernal chest pain. Physical examination revealed icterus. Examination of the abdomen did not reveal any organomegaly. Liver function test revealed a predominantly conjugated bilirubinemia. Abdominal sonogram revealed thrombosis and occlusion of the posterior right portal vein. Liver parenchyma was homogenous with no intrahepatic mass. Computed tomography (CT) of the abdomen and pelvis after administration of oral and intravenous contrast with delayed views revealed arterial enhancement of the right portal vein thrombus with delayed washout. MRI of the abdomen with gadolinium confirmed the right portal vein thrombus without focal hepatic mass. Aspiration of the right portal vein thrombus under CT guidance revealed hepatocellular carcinoma which was confirmed by immunohistochemistry. Serum alpha-fetoprotein level was very high. Patient was started on sorafenib with subsequent decrease in alpha-fetoprotein level. He was doing well till the date of this report.DiscussionThis unusual case of hepatocellular carcinoma presenting as an incidental malignant portal vein thrombosis without any primary liver lesion is extremely rare. Other reported cases of malignant portal vein thrombosis have been in patients with underlying hepatoma, cirrhosis, or with intrabiliary hepatocelluar carcinoma. In the clinical setting of portal vein thrombosis, imaging studies showing enhancement of the thrombus in the arterial phase are important in leading to the diagnosis of malignancy.

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/mm3 with 24 k/mm3 band neutrophils, and absolute neutrophil count of 69 k/mm3. 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 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.

Extravascular hemolysis mimicking severe obstructive jaundice

Most commonly direct hyperbilirubinemia is secondary to bstruction of hepatobiliary, pancreatic, or intestinal source. nly four reported cases describe marked direct hyperbiliruinemia from extravascular hemolysis after massive packed red lood cell transfusion [1]. The labs imitated obstructive jaundice ith workup and autopsy negative for obstruction. A 92-year-old-man was admitted to the emergency room after fall. Exam revealed a deformed right upper arm. Labs revealed: hemoglobin 12.4 g/dl, total bilirubin .9 mg/dl, alkaline phosphatase 107 units/L. CT-scan showed right humeral fracture and pelvic fracture with hematoma. e went for open reduction and internal fixation of the right umeral fracture opting for conservative management of the elvic fracture. Severe anemia secondary to a subcutaneous and etroperitoneal bleed required 14 units of packed red blood cells, 4 units of fresh frozen plasma, and two units of platelets. His otal bilirubin trended upward reaching 33.5 mg/dl with a direct raction of 27.3 mg/dl. Hemolytic workup revealed an LDH f 237, haptoglobin level of 48, and negative direct Coomb’s. he peripheral smear showed no fragmentation. He developed arked jaundice and a large hematoma on his left thoracic cavty. Repeat CT on day 8 revealed an extensive subcutaneous and etroperitoneal hematoma involving left thoracic and abdominal all. Abdominal ultrasonography was normal. Hepatobiliary minodiacetic acid (HIDA) scan performed showed uniform epatic uptake and excretion with normal visualization of the ommon bile duct, gall bladder, and small bowel. Viral hepatiis, autoimmune hepatitis, ceruloplasmin, alpha-1-antitrypsin, emochromatosis panel were unremarkable. The patient’s course was complicated by pneumonia and ventual respiratory failure with the family opting for comfort are. Extravascular destruction of heme in subcutaeous/retroperitoneal hemorrhage sites by phagocytic cells s the likely explanation for the direct hyperbilirubinemia. vidence suggests heme may be degraded by pathways other han the biliary pathway. 35 mg of bilirubin should be produced rom degradation of 1 g of hemoglobin. However, recovery f fecal urobilinogen is substantially less suggesting that 0 to 40% of heme may be degraded by another pathway 2]. In complete biliary obstruction excretion of conjugated F d T f

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 variants 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 cells 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.

Germ cell cancer presenting as gastrointestinal bleeding and developing brain metastases: case report and review of the literature

This paper describes a rare case of germ cell cancer with duodenum, brain and lung metastases. The patient presented with melena and left testicle enlargement. Orchiectomy revealed mixed germ cell cancer, enteroscopy revealed duodenal choriocarcinoma, and chest x-ray and computed tomography (CT) showed bilateral lung metastases. The patient received and tolerated cisplatinum-based chemotherapy, and responded well. However, he developed seizures 3 months later. MRI showed brain metastases and he was treated with whole-brain radiation. One month later, he developed progressive dyspnea. Chest CT showed worsening lung metastases. He received second-line chemotherapy, but died due to multiorgan failure. Germ cell cancer with nonpulmonary metastases has poor prognosis and the management of these patients requires a multimodal approach. Head CT should be considered as routine screening for all germ cell cancer patients on initial diagnosis and brain MRI should be considered for high-risk patients (with an embryo- or choriocarcinoma histology, dramatically elevated β-human chorionic gonadotropin and lung involvement).

Hairy cell leukemia in a patient with situs inversus totalis: an extremely rare combination

Hairy cell leukemia is a rare cancer of the blood. The occurrence of hairy cell leukemia with another very rare genetic disorder makes us question whether it is just a coincidence. This article reports the first case of hairy cell leukemia in a patient with situs inversus totalis in western literature. There have been studies into the pathogenesis of situs inversus totalis that suggest it is caused by the failure of embryonic cells to properly rotate during embryogenesis. On the molecular level, the nodal cilia, which are responsible for embryonic rotation, are built by transport through the KIF3 complex - a kinesin superfamily of molecular motors. The KIF3 complex is also responsible for N-cadherin movement in cells. Furthermore, it is well known that these cell adhesion molecules play an important role in carcinogenesis and its progression. This report attempts to link the rare conditions and propose a possible genetic relationship between the two.