Meghana Bansal

Ipilimumab-induced necrotic myelopathy in a patient with metastatic melanoma: A case report and review of literature

Ipilimumab is a novel humanized monoclonal antibody directed against cytotoxic T lymphocyte antigen 4, a T-cell surface molecule involved in down-regulation and suppression of the T cell response to stimuli. Patients treated with ipilimumab are at risk for immune-related adverse events involving the skin, digestive tract, liver and endocrine organs. Few case reports of immune-related adverse effects involving central or peripheral nervous system due to ipilimumab are published. These include inflammatory myopathy, aseptic meningitis, severe meningo-radiculo-neuritis, temporal arteritis, Guillain-Barre syndrome, and posterior reversible encephalopathy syndrome. We report the first case of ipilimumab-induced progressive necrotic myelopathy.

A rapidly progressing Pancoast syndrome due to pulmonary mucormycosis: a case report

IntroductionPancoast syndrome is characterized by Horner syndrome, shoulder pain radiating down the arm, compression of the brachial blood vessels, and, in long-standing cases, atrophy of the arm and hand muscles. It is most commonly associated with lung carcinoma but rarely is seen with certain infections.Case presentationWe present the case of a 51-year-old Caucasian man who had acute myeloid leukemia and who developed a rapidly fulminating pneumonia along with signs and symptoms of acute brachial plexopathy and left Horner syndrome. Also, a purpuric plaque developed over his left chest wall and progressed to skin necrosis. The skin biopsy and bronchoalveolar lavage showed a Rhizopus species, leading to a diagnosis of mucormycosis. This is a rare case of pneumonia due to mucormycosis associated with acute Pancoast syndrome.ConclusionsAccording to our review of the literature, only a few infectious agents have been reported to be associated with Pancoast syndrome. We found only three case reports of mucormycosis associated with acute Pancoast syndrome. Clinicians should consider mucormycosis in their differential diagnosis in a patient with pulmonary lesions and chest wall invasion with or without neurological symptoms, especially in the setting of neutropenia or other immunosuppressed conditions. It is important to recognize this condition early in order to target therapy and interventions.

Ectopic Adrenocorticotropic Hormone Syndrome Due to a Pancreatic Neuroendocrine Tumor.

Ectopic adrenocorticotropic hormone (ACTH) secretion can occur due to a spectrum of lesions, from occult undetectable lesions to aggressive, metastatic tumors. Ectopic ACTH syndrome due to a pancreatic neuroendocrine tumor (PNET) is very rare. Patients with this tumor usually develop early metastases, even prior to symptoms and have a poor prognosis. We present a woman with ACTH-secreting PNET with liver metastasis who presented with new-onset diabetes, hypertension, and some features of Cushing’s syndrome associated with weight loss, which were reversed with resection of the tumor. We summarize the treatment advances in the management of PNETs.

A unique presentation of unilateral pleural effusion in a patient with a high-grade plasma cell neoplasm

High-grade plasma cell neoplasms include multiple myeloma with plasmablastic morphology, primary effusion lymphoma (PEL), and plasmablastic lymphoma (PBL) [1]. Although these entities have specific diagnostic criteria in the World Health Organization (WHO) Classification [1], there may be some overlap in the clinical presentation of these diseases. Furthermore, occasional rare patients present with highgrade plasma cell neoplasms that do not fit well into any defined category. Here we describe a case of a high-grade plasma cell neoplasm in a HIV-negative, elderly patient with a unique presentation of unilateral pleural effusion preceding development of lymphadenopathy. This case illustrates the challenges that may occur in the diagnosis and treatment of an atypical high-grade plasma cell neoplasm. The patient was a 70-year-old man with a history of stage IV diffuse large B-cell lymphoma (DLBCL) diagnosed in July 2006 when he was 63 years old. Malignant B cells had centroblastic morphology, expressed CD20, BCL2 and BCL6 without CD138 expression, and had a hyperdiploid karyotype with multiple structural and numerical abnormalities. Fluorescence in situ hybridization (FISH) analysis revealed 3-6 non-rearranged MYC gene signals that were consistent with 3-6 copies of chromosome 8 seen in karyotype. The patient received eight cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy and remained in complete remission until February 2014, at which time the patient presented to the hospital with shortness of breath and left sided pleuritic chest pain. He denied hemoptysis, cough, fever, chills, rigors, night sweats, weight loss or loss of appetite. Chest X-ray (CXR) revealed interval development of a moderate left pleural effusion, and subsequent computed tomography (CT) scan of the chest and abdomen showed a large left pleural effusion with near complete atelectasis of the left lower and lingual lobes [Figure 1(A)]. There was no interval development of lymphadenopathy in the chest, abdomen or retroperitoneum and no lymphadenopathy in the supraclavicular and axillary regions. A therapeutic and diagnostic thoracentesis showed a heavily blood stained pleural effusion with lactate dehydrogenase (LDH) 3120 IU/L, white blood cells (WBC) 18 600/mm3 and red blood cells (RBC) 390 000/mm3. Bacterial culture of the fluid was negative. Cytologic smear of the fluid showed a large population of malignant cells with plasmacytic/plasmablastic features [Figure 1(B)]. Immunohistochemical staining showed that the malignant cells were positive for CD138, but negative for CD20, PAX-5, BCL2, BCL6, cytokeratin AE1/AE3, S-100, CK5/6 and TTF-1 (data not shown). The malignant cells were negative by immunohistochemistry for human herpesvirus 8 (HHV8) and negative by in situ hybridization for EBV-encoded RNA (EBER). Flow cytometry of the pleural fluid showed that the malignant cells were positive for CD38, CD56, CD138 and CD79b, but negative for CD3, CD5, CD10, CD19, CD20, CD22, CD30, CD34 and CD45. FISH analysis revealed that the malignant cells were positive for two different IGH rearrangements, FGFR3-IGH and MYC-IGH, with an extra chromosome 1q signal. Malignant cells were negative for CCND1–IGH or IGH–MAF fusions and negative for BCL2 gene rearrangement. Cytogenetic analysis of the pleural fluid revealed a complex hypodiploid clone in all cells analyzed [45,X,  Y, add (1)(p12), add (1)(q25), add (2)(p16), del (5)(q31),  7,  8, add (10) (q24),  13, del (13;14)(q10;q10), add (15)(q26), add (17) (q21),  18,  3mar] not clonally related to the DLBCL diagnosed in 2006. Laboratory values showed a normal complete blood count (CBC), normal serum creatinine, calcium and LDH. Serum protein electrophoresis (SPEP) showed a faint M spike identified by immunofixation electrophoresis (IFE) as immunoglobulin G (IgG) kappa, with a serum free kappa light chain of 41.8 mg/dL, serum free lambda light chain of Leukemia & Lymphoma, October 2015; 56(10): 2989–2991

Unusual Enhancing Foci

and lower gastrointestinal endoscopies, a bone scan, and serum analysis for tumor markers, was negative. Over the course of the next 3 years, follow-up CT scans had shown slow enlargement of the mass. Four weeks prior to the current visit, the patient had presented with subjective fevers, anorexia, and right upperquadrant abdominal pain of 3 months duration, as well as a gradual weight loss of 40 pounds over the preceding 2 years. He had no chest pain, cough, melena, hematochezia, or change in bowel habits. He used chewing tobacco and had family history (brother) of lung cancer. An abdominal CT scan during that visit had shown a further increase in the size of the mass. A positron emission tomography (PET) scan showed increased glucose uptake in the mass inferolateral to the right lobe of the liver, as well as in the ascending colon, bilateral hilar lymph nodes, and anterior abdominal wall, consistent with peritoneal metastases (Figure 2). Fine-needle aspiration cytology of the mass had shown only non-specific inflammatory cells, but the night sweats, anorexia, weight loss, and slow tumor enlargement seemed to suggest a recurrence of the gastrointestinal stromal tumor, and imatinib chemotherapy had been initiated.

A liver lesion with systemic inflammatory manifestations

DOI: 10.4103/1319-3767.98441 A 50-year-old Caucasian man presented with recurrent fevers, anorexia, and 15 kg weight loss over 10 weeks. He had no abdominal pain, jaundice, or bleeding in stools. He did not smoke or drink alcohol. On physical examination, his abdomen was soft with an enlarged nontender liver. Laboratory workup showed a white cell count of 6800/mm3, anemia with hemoglobin of 9.4 g/dL, and elevated platelets of 466,000/mm3. Sedimentation rate (ESR) was greater than 140 mm/h and C-reactive protein was 76 mg/dL. Serum iron and transferrin was low with an elevated ferritin consistent with anemia of chronic disease. Liver function tests were normal except for an elevated alkaline phosphatase of 178 U/L. Computed tomography of the abdomen [Figure 1] showed a 20 cm lesion with peripheral and discontinuous nodular enhancement within the majority of the right lobe of the liver. Magnetic resonance imaging [Figure 2] showed a large heterogeneous lesion of 20 cm occupying almost the entire right lobe of the liver. There was hyperintense signal on T2-weighted images with discontinuous centripetal peripheral nodular enhancement. The lesion also had hypointense areas compatible with hemorrhage.

Biliary Actinomycosis Mimicking a Klatskin Tumor

A 59-year-old White woman presented with painless jaundice, pruritus, and 10-lb weight loss over a month to an outside facility. She did not have any fever. There was no past history of pancreatitis, abdominal surgery, or trauma. She had no history of smoking. Her labs were: total bilirubin 11.0 mg/dL, direct bilirubin 10.2 mg/dL, alkaline phosphatase (ALP) 655 U/L, aspartate aminotransferase (AST) 92 U/L, and alanine aminotransferase (ALT) 96 U/L. Magnetic resonance imaging showed a 4.5-cm mass at the porta hepatis with compression of the common bile duct and portal lymphadenopathy. CA 19-9 was elevated at 96 U/ml. She underwent a placement of two external biliary drains and had resolution of the jaundice. She was presumed to have Klatskin tumor, based on her clinical and radiological presentation, and the mass was unresectable given its proximity to the portal vein and hepatic artery. She was referred to our center for medical management of cholangiocarcinoma. Patients initially choose to be palliative and refused biopsy considering the high mortality of the tumor. She presented a few days later with abdominal pain and agreed for a biopsy. A few days after a computed tomography (CT)-guided biopsy of the mass, she developed fever, chills, hematemesis, and abdominal pain. On examination, she had a temperature of 99.6°F, blood pressure of 105/60 mmHg, pulse of 105/min, and respiration of 20/min. Her lungs were clear to auscultation. She had tenderness in the right upper quadrant of the abdomen. A repeat CT scan showed a 4.7-cm porta hepatis mass with encasement of the hepatic artery and with loss of fat planes between the mass and the gall bladder and duodenum (Fig. 1). A 1.7-cm gall stone was visualized. A 1-cm gastrohepatic lymph node was seen along with scattered lymph nodes measuring up to 1 cm in the retrocaval, aortocaval, and periaortic regions. Two biliary stents were in place. Upper gastrointestinal endoscopy showed fresh blood oozing from the ampulla of Vater, which was felt to be due to her mass. Digital subtraction angiography of the celiac trunk and superior mesenteric artery showed an 8-mm pseudoaneurysm of the third segmental branch of the left hepatic artery (Fig. 2). The bleeding was successfully stopped with coil and gel foam embolization. The pathology of the bile duct mass was negative for malignancy but showed acute and chronic inflammation and granulomas with gram-positive filamentous bacteria consistent with actinomycetes species (Fig. 3). Cultures grew Streptococcus constellatus, Prevotella intermedia, and Prevotella oris. The patient was started on IV penicillin, oral metronidazole and levaquin while in the hospital and made a slow recovery. M. Bansal (*) :A. Agarwal Department of Internal Medicine, University of Arkansas for Medical Sciences, 4301 W Markham St, Little Rock, AR 72205, USA e-mail: mbansal@uams.edu