Granulocytic sarcoma of the pancreas: a report of two cases and literature review

Granulocytic sarcomas (GS) are extramedullary tumour masses of immature myeloid cells, also known as chloroma and extramedullary myeloid cell tumour. These neoplasms usually occur simultaneously with, or follow the onset of, acute myeloid leukaemia (AML). Rarely, they are the first manifestation of AML. GS may also be the first sign of transformation to AML in patients with chronic myeloproliferative disorders and myelodysplastic syndromes. GS have been reported to occur in a variety of tissues, but presentation as an abdominal mass and, in particular, infiltration of the pancreas is rare. We report two cases of pancreatic GS, review the literature, and discuss recent insights into the basic biological properties of these rare tumours.     

Granulocytic sarcoma of the pancreas. A report of one case and review of the literature

Granulocytic sarcoma is an extramedullary collection of myeloblasts. Granulocytic sarcomas usually arise during the course of acute myeloid leukemia, although they also occur infrequently in chronic myeloid leukemia and other myeloproliferative disorders. We are reporting a very unusual presentation of granulocytic sarcoma in a patient with poorly differentiated (FAB type M2) acute myeloid leukemia. The patient was in complete remission from leukemia when he presented obstructive jaundice due to a pancreatic mass consisting of myeloblasts. A literature search identified only five previously reported cases of granulocytic sarcoma of the pancreas. Those cases are also reviewed here with emphasis on their clinical characteristics, diagnostic approaches, and management of this interesting and challenging entity.     

Granulocytic sarcoma of the pancreas: A case report and review of the literatures

Background  

Granulocytic sarcoma (GS) is a form of acute myeloid leukemia (AML), also known as extramedullary myeloid tumor or chloroma. It forms a solid malignant tumor consisting of myelocytes or granulocytes and is typically located in bone while occurrence in other parts of the body is rare.     

Tuberculosis of the pancreas: report of two cases and review of the literature

Tuberculosis of the pancreas is a clinical entity rarely described in the literature. The pancreas is biologically protected from infection by Mycobacterium tuberculosis, probably because of the presence of pancreatic enzymes that interfere with the seeding of M. tuberculosis. However, when pathogens are able to overcome the resistance, they can have diverse presentations, such as pancreatic masses that can mimic carcinoma, obstructive jaundice, pancreatitis, and gastrointestinal bleeding. Herein we describe 2 cases of pancreatic tuberculosis that presented as multicystic masses, and we review the literature to describe the diverse clinical manifestations of this condition.     

Annular pancreas in adults. A report of two cases and a review of the literature

Annular pancreas is an uncommon developmental abnormality of the ventral pancreas. Half of all patients present in adulthood. Abdominal pain and duodenal obstruction are the commonest presenting features. Despite newer imaging modalities, the diagnosis is mostly made at surgery. We report on two patients and review the embryology, presentation, diagnosis and the current surgical management options of annular pancreas.     

Myeloid sarcoma of the pancreas: A case report and literature review

Introduction:Myeloid sarcoma (MS) is an extramedullary mass, consisting of myeloid blasts with or without maturation, which efface the normal tissue architecture. It occurs mainly in lymph nodes, skin and soft tissue, testis, bone, peritoneum, and gastrointestinal tract, but rarely in the pancreas. Because their clinical courses, treatments, and prognoses are quite different, it is crucially important to distinguish between MS and pancreatic cancer.Patient concerns:We herein report a rare case of acute myeloid leukemia (AML) which presented with a pancreatic mass that mimicked pancreatic cancer.Diagnosis: The diagnosis of MS was established based on immunohistochemical (IHC) analysis and bone marrow examination which revealed neoplastic cells with CD34+/CD117+.Interventions:The patient was actively treated with chemotherapy.Outcomes:After 4 cycles of chemotherapy, the lesion in pancreas was significantly reduced, and the patient is still receiving further chemotherapy.Conclusion:When we encounter a patient suspected of pancreatic cancer with blood cell abnormalities and no significant increase in carbohydrate antigen 19-9 (CA19-9), we need to be aware of the possibility of pancreatic MS. Preoperative pathological biopsy and IHC are indispensable. Misdiagnosis is common if we rely solely on imaging.     

Double pylorus: report of two cases and review of the literature.

Double pylorus is a rare condition consisting of a double communication between gastric antrum and duodenal bulb; in most cases it is a complication of penetrating ulcer, sometimes it is a congenital abnormality. The prevalence of this rare anomaly ranges from 0.02% to 0.13%; the male:female ratio is about 2:1. Two cases of acquired double pylorus are reported with a review of the literature. The first case represented an occasional report; in the other one the development of double pylorus from confluent prepiloric and bulbar ulcers was documented through serial endoscopies. Both patients were affected with chronic renal failure and referred previous treatment with diclofenac; however, their relationship with double pylorus onset remains unproven.     

Follicular dendritic cell sarcoma: a report of 14 cases and a review of the literature

Follicular dendritic cell sarcomas (FDCS) are grouped with the histiocytic and dendritic cell neoplasms. The natural history and response to different treatments have not been well established. The cases of 14 patients with FDCS who were seen at M. D. Anderson between 1995 and 2005 were reviewed. Median patient age was 48 years (range, 25-69 years). Histologically, four cases showed low-grade features, three cases showed low-grade features with focal high-grade features, and five cases showed high-grade features. Tumors were positive for CD21, CD23, and CD35 in 83, 90, and 44% of cases, respectively. Twelve (92%) of 13 tumors were strongly positive for epidermal growth factor receptor. Information on initial treatment was available in 11 patients, which included surgery alone in one patient, surgery and radiation in two, surgery and chemotherapy in one, chemotherapy alone in three, chemotherapy and radiation in one, surgery followed by radiation and chemotherapy in three patients. In eight patients the initial chemotherapy regimen was cyclophosphamide, doxorubicin, vincristine, and prednisone. Complete remission was achieved in 7 (63%) of 11 patients. Ten patients were alive at a median follow up of 22 months, 3 (23%) of 13 had no evidence of disease, and 7 (53%) of 13 patients were alive with disease. Follicular sarcoma is an aggressive neoplasm. Although most of the patients initially responded to treatment, the majority of them (81%) relapsed. A better understanding of the biology of FDCS could guide our efforts in the development of new treatment modalities for this rare disease.     

Superwarfarin poisoning: a report of two cases and review of the literature

Superwarfarins are anticoagulant rodenticides similar to warfarin, but which have various substituted phenyl groups replacing the terminal methyl group, resulting in a fat-soluble, long-acting anticoagulant that is nearly 100 times more potent than the parent compound. Since their development, many accidental and intentional cases of consumption have been reported. We describe two cases of consumption, one related to unknown etiology, and the other related to utilization of the superwarfarin to potentiate a drug of abuse. The clinical manifestations including bleeding symptoms and abnormal coagulation assays are discussed. The differential diagnosis is quite broad, and includes all causes of vitamin K deficiency, factor deficiency or inhibitor, disseminated intravascular coagulation (DIC), and liver disease. Differentiating superwarfarin ingestion from the other causes can be quite difficult, but extremely important, as management requires prolonged administration of vitamin K. Other treatment options are discussed as well including, fresh frozen plasma (FFP), and recombinant factor VIIa. Finally, the significance of "lacing" drugs of abuse with superwarfarin to potentiate their effect is discussed, as well as the complications that could develop from such a habit.     

食管气囊肿症2例

气囊肿症(pneumatosis cystoides,PC)是指胃肠道黏膜下层薄壁气囊肿为特征的病理改变,又称囊样积气症,多见于结肠和小肠,胃和食管较为罕见.本文报道病例1为咳嗽伴上腹痛,病例2为食管异物后患者,内镜检查时偶被发现;临床无特异性表现,治疗原发病,局部未予特殊和手术治疗,2 wk后内镜随访观察气囊样改变消...     

Cardiac hemangioma: a report of two cases and review of the literature

Cardiac hemangioma is extremely rare. We encountered two patients with cardiac hemangioma detected by thoracic echocardiography during a medical checkup. In the first case, transthoracic echocardiography revealed a pedunculated tumor in the left ventricle. Selective left coronary angiography demonstrated that the main feeding artery of the tumor arose from the third diagonal branch of the left anterior descending coronary artery. In the second case, thoracic and transesophageal echocardiography showed an oval tumor arising from the right atrium. Both tumors were successfully resected. Histopathological examination revealed that one of the tumors was a capillary hemangioma and the other was a mixed capillary and cavernous hemangioma. After operation, both patients had an uneventful recovery without any complications.     

Esophageal xanthoma: report of two cases and a review of the literature

So far, three cases of esophageal xanthoma have been reported. We describe here endoscopic, microscopic and immunohistochemical findings of two new cases of this rare condition, and a review of five cases of esophageal xanthoma, including our cases. Esophageal xanthomas endoscopically show yellow granular spots or a slightly elevated lesion, and are similar to an ectopic sebaceous gland. Microscopically, an aggregate of foamy histiocytes (lipid islands) is seen immediately beneath the squamous epithelium, particularly between the rete ridges. Immunohistochemically, xanthoma cells are positive for CD68, which indicate a histiocytic origin. The etiology of esophageal xanthoma remains unclear. We emphasize that esophageal xanthomas may have been erroneously diagnosed as an ectopic sebaceous gland on endoscopic examination.     

Primary cardiac sarcoma: report of 3 cases and review of the literature

Primary cardiac sarcoma is a rare tumor with a poor prognosis. We report 3 cases with a review of literature about this disease. There were 2 males and 1 woman. The main symptoms were thoracic pain. The clinical features were various and the thoracic ultra sonography exam allowed the diagnosis in the 3 cases. All patients had surgical remove of their cardiac tumor followed by chemotherapy. All of them died within 13 to 36 months after the diagnosis. Primary cardiac sarcoma has a poor prognosis with a mean survival less than 12 months.     

Congenital short pancreas: a report of two cases.

We report two adult cases with partial agenesis of the pancreas. Their radiological features, including endoscopic retrograde pancreatography are demonstrated. The significance of developmental anomalies involving the pancreas is emphasised.     

Syngeneic graft-versus-host disease: a report of two cases and literature review

Rappeport et al first reported the clinical syndrome of graft-versus-host disease (GVHD) in syngeneic bone marrow transplant patients. Recently, there have been more reports of a GVHD-like syndrome in syngeneic bone marrow transplant patients (SGVHD) that may result in significant clinical morbidity. A total of 17 cases of SGVHD in syngeneic bone marrow transplant patients have been reported to date in the medical literature. The current report reviews these cases and presents two additional cases of severe SGVHD that have occurred at our institution.     

Treatment of ruptured undifferentiated sarcoma of the liver in children: a report of two cases and review of the literature

Undifferentiated (embryonal) sarcoma of the liver (USL) is a highly malignant tumor of early life. Treatment choices for USL, especially with intraperitoneal rupture, are uncertain. Outcomes have been almost uniformly poor until recently. We describe two 7-year-old girls treated for ruptured USL. In the more recent patient, operative biopsy was followed by three cycles of cisplatin (CDDP), adriamycin (ADR), and cyclophosphamide (CPM). A fluid-filled cavity in the tumor showed enlargement and was drained. Two cycles of CDDP, ADR, vincristine (VCR), and ifosfamide were accompanied by reduction in tumor size, and trisegmentectomy was performed. She has no evidence of disease 3.5 years after surgery. In the other patient, left lobectomy was followed by a less intensive regimen, including CPM, VCR, and fluorouracil. This patient died of dissemination within 5 months. In 170 reported pediatric patients with USL, the 2-year disease-free survival was 17%. For the 96 such patients reported since 1980, 2-year disease-free survival had improved to 27%. More aggressive chemotherapy has been associated with this change. Of 8 patients with tumor rupture whose details have been reported (including the 2 present patients) after resection of the tumor, 4 died, 1 was alive with disease, and 3 were free of disease at 8, 49, and 58 months, respectively, after diagnosis. Ruptured USL should be treated with combination chemotherapy including CDDP and ADR, as well as with curative resection.     

Autoimmune pancreatitis:report of two cases and literature review

BACKGROUND:Autoimmune pancreatitis(AIP)is a unique chronic inflammation of the pancreas in which autoimmune mechanisms are involved in the pathogenesis.It is characterized by clinical,histopathological,radiographic,serologic and therapeutic features.Since it was first described in 1995,increasing numbers of AIP patients have been diagnosed.METHODS:In this study,the data from 2 patients with AIP who had elevation of serum immunoglobulin G,positive autoantibody,swelling of the pancreas and narrowing of the ma...     

Retrocaval ureter: report of two cases and literature review

Retrocaval ureter is a rare congenital anomaly in which the ureter passes behind, and is compressed by, the inferior vena cava. Its etiology is assumed to be abnormal embryologic development of the inferior vena cava as a result of atrophy failure of the right subcardinal vein in the lumbar portion. We report two cases of retrocaval ureter and review the relevant literature. One patient was a 7-year-old boy who presented with right flank pain. The other was a 40-year-old male who was found to have right hydronephrosis accidentally on abdominal sonography during a health examination. Both underwent retrograde pyelography which showed the typical S-shape of a retrocaval ureter. Abdominal computerized tomography and magnetic resonance imaging confirmed the diagnosis of retrocaval ureter. Ureteroureterostomies were performed. One patient showed focal squamous metaplasia of the ureter and the other had chronic inflammation and fibrosis. Follow-up studies showed improvement in hydronephrosis and renal function in both patients. We conclude that retrocaval ureter is a rare disorder and surgical correction is usually effective.