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/mm3with 24 k/mm3band 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.

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).

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.

4775 Abdominal pain is a common complaint among all ages of patients. Splenic infarction and splenic vein thrombosis are rare causes of abdominal pain, usually presenting as left-sided abdominal pain associated with fever, nausea or vomiting, and elevated white blood cell count. CT scan is currently the preferred diagnostic test. Once the diagnosis is identified, the etiology of splenic infarction and/or splenic vein thrombosis should be elicited. Retrospective chart review was conducted in all the patients admitted to our hospital from 2000 till now. Four cases of splenic infarction and/or splenic vein thrombosis were identified (4 males, average age of 45 years, range from 38 to 52 years). Case 1, a 45-year-old male with sickle cell trait (HbS of 38.7%), presented with left upper quadrant pain after 5-hour flight and alcohol binge prior to flight. CT showed splenic infarct and splenic vein thrombosis. Patient received aggressive hydration, abdominal pain resolved and was discharged home. Case 2, a 52-year-old male with renal cell carcinoma, presented with generalized abdominal pain. CT showed splenic infarct. Patient was treated with Coumadin, and routinely followed-up at Oncology clinic. Case 3, a 38-year old male with alcohol abuse and chronic pancreatitis, presented with recurrent abdominal pain and hypersplenism. CT showed splenic vein thrombosis, and the patient underwent splenectomy. Case 4, a 45-year-old male with acute pancreatitis, presented with epigastric pain. CT showed splenic vein thrombosis, and the patient was treated with Coumadin. It is important to reveal the underlying causes for splenic infarction and/or splenic vein thrombosis. Splenic infarction can occur in a variety of settings, including hemoglobinopathy (especially sickle cell disease), hypercoagulable state, embolic disease, malignancy and myeloproliferative disorders. Pancreatitis and pancreatic cancer are the most common causes of splenic vein thrombosis. In general, splenic infarction and/or splenic vein thrombosis can be managed safely with medical treatment, including hydration, oxygenation and pain management. Coumadin can be considered in cancer patients with splenic infarction and in patients with splenic vein thrombosis. Splenectomy is indicated in patients with hypersplenism, splenic sequestration crisis, splenic abscess, splenic rupture, and massive splenic infarction. Splenic infarction in sickle cell disease is usually small and repetitive, leading ultimately to autosplenectomy. Splenic infarction in sickle cell trait is rare. High altitude, vigorous exercise, airplane flight, coexistence with thalassemia or hereditary spherocytosis or severe pyruvate kinase deficiency, can precipitate infarction. Interestingly in our first case, the patient with sickle cell trait developed splenic infarction and non-occlusive thrombus in the distal splenic vein after 5-hour flight. It is possible that the hypoxia associated with the commercial flight caused conformational changes in sickle cells, leading to red blood cells sluggish in the splenic red pulp, and eventually leading to splenic infarction. The alcohol binge resulted in dehydration and hemostasis, leading to splenic vein thrombosis, further perpetuating the vicious cycle. Therefore, we recommend adequate hydration and in-flight oxygen supplementation for sickle cell trait patients taking airplane flight. Further studies need to be done to confirm our hypothesis. Disclosures: No relevant conflicts of interest to declare.

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.