Pancreatic endometrioma : a rare differential diagnosis for a pseudocyst.

Pancreatic endometriosis is extremely rare with only 14 cases reported in the medical literature and its diagnosis on radiological imaging poses a great challenge. We report a case of a 31-year-old female patient with recurrent admissions for pancreatitis of unknown aetiology and no relevant previous medical history. Sectional imaging showed a cystic lesion in the tail of the pancreas and the diagnosis of a post-pancreatitis pseudocyst or a less likely pre-malignant mucinous cystadenoma was considered. On post-robotic resection of the pancreatic cyst, the histopathology analysis was positive for endometrial stroma. Pancreatic endometriosis although rare should be considered as a differential diagnosis for cystic lesions especially in patients who are known to have pelvic endometriosis. Nevertheless, the gold standard for the definite diagnosis of pancreatic endometriosis remains histopathological.

A Tangled Autoimmune Trio: Multiple Sclerosis, Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antineutrophil Cytoplasmic Antibody Vasculitis.

The coexistence of multiple autoimmune diseases in the same individual is unusual and has received little attention in the literature. We present a young female patient with multiple sclerosis, systemic lupus erythematosus, and biopsy-proven renal proteinase 3 antineutrophil cytoplasmic antibodyassociated vasculitis who responded well to intravenous rituximab clinically and serologically.

Pancreatic schwannoma: a rare differential diagnosis for a pancreatic mass.

Pancreatic schwannomas are rare benign tumors with low malignant potential and are often difficult to diagnose due to their non-specific presenting symptoms and overlapping radiological imaging characteristics. Cross-sectional imaging plays an important role in the initial diagnosis and in delineating the extent of the lesion. However, biopsy and histopathological examination remains the gold-standard for a definite diagnosis. The management of pancreatic schwannomas includes surgical resection often yielding excellent clinical outcomes with low recurrence rates. We present a case of a 33-year-old female patient with a history of a recurrent vague upper abdominal pain where CT of the upper abdomen showed a hypodense pancreatic mass. Robotic subtotal pancreatectomy was done with histopathology showing spindled Schwann cells indicative of a pancreatic schwannoma.

Clear cell carcinoma of the ovary mimicking struma ovarii and carcinoid tumor.

Clear cell carcinomas are considered as high-grade tumor often with poor prognosis. We describe 2 cases of clear cell carcinomas of the ovary mimicking benign or less aggressive tumors encountered in the female genital track. The first case is mimicking a benign monodermal teratoma, the so-called struma ovarii, and the second mimicking a carcinoid tumor.

Primary Central Nervous System Lymphoma in a Patient on Adalimumab Therapy for Chronic Plaque Psoriasis.

BACKGROUND: Adalimumab (Humira) is a recombinant human monoclonal antibody against tumor necrosis factor alpha, which works by blocking the interaction of tumor necrosis factor alpha with its cell-surface receptors, thereby limiting the progression of inflammatory pathways. Its use is approved for several autoimmune conditions, including chronic plaque psoriasis, for which it has been prescribed as a first-line biologic treatment. Increased risks of malignancy, particularly nonmelanoma skin cancer and non-central nervous system lymphomas, have been reported with use of this drug; however, there have been no reports of central nervous system lymphomas. CASE DESCRIPTION: A 43-year-old man presented for evaluation following recent speech difficulty and a generalized tonic-clonic seizure. His medical history was significant for plaque psoriasis, for which he had been receiving treatment with adalimumab for 4 months. Magnetic resonance imaging scan of the brain with contrast agent showed a well-defined rounded enhancing lesion in the left temporal lobe with circumferential vasogenic edema. Mass effect was noted. Computed tomography scan of the chest, abdomen, and pelvis was unremarkable. He underwent excisional biopsy, and the preliminary intraoperative pathology report revealed a diagnosis of high-grade lymphoma. Subsequent analysis of morphology and immunophenotyping was consistent with primary diffuse large B-cell lymphoma of the central nervous system. Use of adalimumab was discontinued. Following combination therapy with high-dose methotrexate and rituximab along with 20 sessions of cranial radiation therapy, the patient was disease-free at 14-month follow-up. CONCLUSIONS: We report the first case to our knowledge showing a possible association of central nervous lymphoma and adalimumab.

Periampullary duodenal schwannoma mimicking ampullary neoplasm.

Schwannomas are neurogenic tumors that arise from Schwann cells in the neural sheath. Gastrointestinal schwannomas occur most often in the stomach, followed by the colon and the rectum. Duodenal schwannomas are rare amongst mesenchymal tumors of the gastrointestinal tract and only a few cases have been reported up to the current date with an incidence of approximately 2%-6%. Duodenal Schwannomas do not have characteristic imaging features thereby cannot be easily differentiated from other submucosal and adjacent extraluminal neoplasms. We present a case of a 76-year old male patient that presented to our hospital with abdominal pain and was diagnosed after an upper gastrointestinal endoscopy with an ampullary duodenal neoplasm that proved to be a periampullary duodenal Schwannoma on histopathology. Duodenal Schwannomas although rare should be considered in the differential diagnosis of ampullary neoplasms.

A case of peribiliary hepatic cysts in a cirrhotic liver: A mimicker of Klatskin Tumor.

Patients with end-stage liver disease may present to healthcare facilities with features of obstructive jaundice and a picture of hilar cholangiocarcinoma on radiological imaging. Careful observation and knowledge of the presence and higher prevalence of peribiliary hepatic cysts in a cirrhotic liver can aid in differentiating this benign entity from malignancy that may halt or delay the patients' eligibility for receiving a liver transplant. We present a case of a patient with liver cirrhosis initially diagnosed as Klatskin tumor on imaging then as a simple case of multiple peribiliary hepatic cysts with the patient eventually undergoing successful liver transplantation.

Fast Track to Accreditation: An Implementation Review of College of American Pathologists and International Organization for Standardization 15189 Accreditation.

CONTEXT: - This review examines challenges and opportunities in preparing laboratories in a startup phase for accreditation by both the College of American Pathologists (CAP) and International Organization for Standardization (ISO) 15189 in an international setting as it relates to our experience at Cleveland Clinic Abu Dhabi Laboratory. It also discusses some of the strategies used in executing those projects and the added advantages in pursuing both types of accreditations. OBJECTIVES: - To share our experience with CAP and ISO 15189 accreditations in a startup international operation in relation to the challenges encountered and implementation strategy success factors. DATA SOURCES: - MEDLINE (PubMed) database was used to review this topic as well as peer-reviewed articles and World Health Organization publications on the topic. CONCLUSIONS: - Accreditation is a perfect means toward building quality medical laboratories in a diverse workforce environment and improving patient safety. Further, it establishes a strong foundation on which any new operation can build a sustainable quality improvement culture. Accreditations by CAP and/or ISO are among the most reputable and well-established accreditation systems that clinical laboratories could aim for. As a result of both accreditations offering synergistic and complementing features, we recommend that any laboratory seeking excellence in quality and performance should consider exploring both. Key elements to success include having dedicated project management and change management support while preparing for accreditation. Laboratories seeking accreditation in early operational stages may face a number of challenges; however, significant opportunities will also be present to optimize various operational components from the beginning.

Constitutive Ras signaling and Ink4a/Arf inactivation cooperate during the development of B-ALL in mice.

Despite recent advances in treatment, human precursor B-cell acute lymphoblastic leukemia (B-ALL) remains a challenging clinical entity. Recent genome-wide studies have uncovered frequent genetic alterations involving RAS pathway mutations and loss of the INK4A/ARF locus, suggesting their important role in the pathogenesis, relapse, and chemotherapy resistance of B-ALL. To better understand the oncogenic mechanisms by which these alterations might promote B-ALL and to develop an in vivo preclinical model of relapsed B-ALL, we engineered mouse strains with induced somatic KrasG12D pathway activation and/or loss of Ink4a/Arf during early stages of B-cell development. Although constitutive activation of KrasG12D in B cells induced prominent transcriptional changes that resulted in enhanced proliferation, it was not sufficient by itself to induce development of a high-grade leukemia/lymphoma. Instead, in 40% of mice, these engineered mutations promoted development of a clonal low-grade lymphoproliferative disorder resembling human extranodal marginal-zone lymphoma of mucosa-associated lymphoid tissue or lymphoplasmacytic lymphoma. Interestingly, loss of the Ink4a/Arf locus, apart from reducing the number of apoptotic B cells broadly attenuated KrasG12D-induced transcriptional signatures. However, combined Kras activation and Ink4a/Arf inactivation cooperated functionally to induce a fully penetrant, highly aggressive B-ALL phenotype resembling high-risk subtypes of human B-ALL such as BCR-ABL and CRFL2-rearranged. Ninety percent of examined murine B-ALL tumors showed loss of the wild-type Ink4a/Arf locus without acquisition of highly recurrent cooperating events, underscoring the role of Ink4a/Arf in restraining Kras-driven oncogenesis in the lymphoid compartment. These data highlight the importance of functional cooperation between mutated Kras and Ink4a/Arf loss on B-ALL.

Severe thrombocytopenia in a patient with hepatitis C treated with eltrombopag from off-label drug use to on-label drug use: a case report.

INTRODUCTION: Off-label drug use refers to drug use beyond the specifications authorized for marketing. Eltrombopag is a new thrombopoietin receptor agonist which was used successfully in this critical case of thrombocytopenia associated with hepatitis C infection before it became an approved drug for such cases. CASE PRESENTATION: A 56-year-old Kuwaiti woman with hepatitis C virus infection was treated with pegylated interferon α-2a and ribavirin, laboratory test results prior to therapy were within normal values. After 4 weeks of that treatment, she developed neutropenia and severe thrombocytopenia. Her hepatitis C virus treatment was stopped for many years until eltrombopag was used as an off-label drug with an episode of severe thrombocytopenia. Her platelets count returned to normal level when triple therapy for hepatitis C virus was used successfully. CONCLUSIONS: An off-label drug should be used only when it is the best available drug, based on evidence from on-going multicenter trials. It could be life saving for some patients in critical situations. However, clinical use of eltrombopag later confirmed that it is a safe and effective drug for immune thrombocytopenic purpura or thrombocytopenia associated with hepatitis C virus infection.

Clear cell carcinomas of the mullerian system: does the pathogenesis vary depending on their nuclear grade and their association with endometriosis? An immunohistochemical analysis.

UNLABELLED: Clear cell carcinomas (CCC) of the mullerian system are considered high grade tumors, but morphologically, the cells of CCC show both low and high grade features. The aims of the current study were to categorize CCC into low and high nuclear grade types, correlate their association with endometriosis, and then observe possible variations in pathogenesis based on their expression of p53 and Ki-67. We studied 41 pure mullerian CCCs and designated each as either a high (HNG) or low (LNG) nuclear grade tumor. Morphologically, 17 (41%) CCCs were LNG and 24 (59%) were HNG. Nine (38%) HNG and 2 (12%) LNG tumors showed positive immunostaining with p53. Endometriosis was associated with 8 (47%) LNG tumors and 8 (33%) HNG CCCs. Of the 11 cases with p53 alteration, 4 (1 LNG and 3 HNG) were associated with endometriosis. CONCLUSIONS: HNG CCCs, irrespective of their association with endometriosis, have alterations of p53. In general, LNG ovarian and endometrial CCCs, irrespective of their association with endometriosis/adenomyosis, are less likely to show p53 alteration. It appears that mullerian CCCs may have variable pathogenesis depending on their nuclear grade and association with endometriosis. A larger study is needed to validate these findings.

CD30-positive EBV-associated diffuse large B-cell lymphoma occurring after immunosuppressive therapy for T-cell prolymphocytic leukemia.

We describe the case of a 64-year-old man who developed diffuse large B-cell lymphoma (DLBCL) in less than a year after he was diagnosed and treated for T-cell prolymphocytic leukemia (T-PLL). At the time of diagnosis of T-PLL he had a white blood cell count (WBC) of 38.2×10(9)/L and only few small lymph nodes were identified on physical examination. Hepatosplenomegaly or skin lesions were not present. Peripheral blood examination was remarkable for 91% circulating prolymphocytes, which by flow cytometry immunophenotypic analysis were CD2, CD3, CD5, and CD7 positive and coexpressed CD4 and CD8 (absolute number, 33.4×10(9)/L). T-cell receptor (TCR) ß and γ genes rearrangements were identified by polymerase chain reaction (PCR). The patient underwent chemotherapy, but did not completely achieve cytogenetic remission. Nine months after his diagnosis of T-PLL, he underwent surgical excision of a new 7 cm left inguinal mass, and was diagnosed with CD30 positive Epstein-Barr Virus (EBV)-associated DLBCL.

Primary follicular lymphoma of the fallopian tube found incidentally in a patient treated for endometrial carcinoma: a case report.

We report a rare case of primary lymphoma of fallopian tube in a 68-year-old woman who underwent total hysterectomy and bilateral salpingo-oophorectomy for endometrial carcinoma. The specimen showed a well-differentiated endometrioid adenocarcinoma with superficial myometrial invasion. The left fallopian tube revealed a 1 cm nodule that histologically showed diffuse lymphoid follicles consisting of small cleaved lymphocytes and occasional larger cells. The cells were immunopositive for CD20, BCL-2, and BCL-6 but negative for CD3 and CD43. Polymerase chain reaction confirmed a monoclonal B-cell population. Fluorescence in-situ hybridization revealed at (14, 18) translocation. The patient had absent lymphadenopathy and negative CT scan of chest, abdomen, and pelvis. The findings were consistent with a primary low grade follicular lymphoma of fallopian tube. She did not receive chemotherapy and remained disease free for 13 months after surgery. Our case suggests that primary lymphoma of fallopian tube may be associated with a favorable prognosis.

Complete remission in two cases of adult T-cell leukemia/lymphoma treated with hyper-CVAD: a case report and review of the literature.

BACKGROUND: Acute T-cell leukemia/lymphoma (ATLL) is a post thymic (peripheral) T-cell neoplasm caused by human T-cell lymphotropic virus type 1 (HTLV-1). Historically, the chemotherapy regimen CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) has been the standard treatment of this rare malignancy. However, its prognosis is poor and median survival in the aggressive variants of ATLL is only 6-10 months. Recently, a more aggressive regimen piloted in Japan, vincristine/cyclophosphamide/doxorubicin/prednisone (VCAP)- doxorubicin/ranimustine/prednisone (AMP)- vindesine/etoposide/carboplatin/prednisone (VECP), has been reported to yield better survival results over biweekly CHOP in a phase III trial. However, the hyper- cyclophosphamide/vincristine/doxorubicin/dexamethasone (CVAD) regimen is a much more frequently used regimen for the treatment of aggressive hematologic malignancies, and has a higher intensity then CHOP. Yet, there is little reported experience with hyper-CVAD regimen in ATLL. CASE REPORTS: We present 2 patients diagnosed with ATLL who were treated with hyper-CVAD chemotherapy and have achieved a durable complete remission. One of the patients has gone on to receive an allogeneic bone marrow transplantation and has been in complete remission for over 18 months. The other has been in a continuous remission for approximately 12 months. We also review the past published experience with the hyper-CVAD regimen in patients with ATLL. CONCLUSION: A commonly used chemotherapy regimen for aggressive hematologic malignancies, hyper-CVAD, can induce durable remissions in patients with ATLL.