Zuzan Cayci

Lacrimal Gland Adenoid Cystic Carcinoma with High-Grade Transformation to Myoepithelial Carcinoma: Report of a Case and Review of Literature

Adenoid cystic carcinoma (AdCC) is the most frequent malignant neoplasm of the lacrimal glands. Like its salivary gland counterpart, lacrimal AdCC can rarely undergo high-grade transformation (“dedifferentiation”). We herein report the clinical, radiographic and microscopic findings of a lacrimal gland AdCC with high grade transformation, occurring in a 39-year-old female patient. In contrast to salivary gland AdCC with high-grade transformation, which usually shows a high grade component with “ductal” differentiation, in the case presented, the “dedifferentiated” component showed morphologic and immunohistochemical features of myoepithelial differentiation.

The use of 18F-FDG PET ratios in the differential diagnosis of common malignant brain tumors.

OBJECTIVE This study examined the use of positron emission tomography (PET) ratios to improve the diagnostic ability of 18F-FDG PET/computed tomography (CT) in the differentiation of enhancing brain metastases, central nervous system lymphomas, and gliomas. MATERIALS AND METHODS Seventy-six patients with malignant brain tumors and available magnetic resonance imaging and PET/CT examinations were included. RESULTS Among standardized uptake value (SUV)-related parameters tested, tumor maximum SUV to ipsilateral cortex maximum SUV ratio (Tmax:WMimax) and maximum SUV (SUVmax) proved to be the two most valuable parameters for differential diagnosis. CONCLUSION In addition to SUVmax, Tmax:WMimax also seems to provide helpful information for the differential diagnosis of lymphomas from other malignant brain tumors.

Allogeneic hematopoietic cell transplantation in systemic mastocytosis: Is there a high risk for veno-occlusive disease?

To the Editor: Advanced systemic mastocytosis (AdvSM) is associated with shortened survival. The only potentially curative therapy in AdvSM is allogeneic hematopoietic cell transplantation (alloHCT) with durable disease-free survival reported in 51% of patients (1). Veno-occlusive disease (VOD) is a known potential complication of alloHCT (2) and can affect liver and less commonly lung. Severe VOD with multiorgan

Differentiation of benign from malignant cervical lymph nodes in patients with head and neck cancer using PET/CT imaging.

PURPOSE To differentiate malignant from benign cervical lymph nodes in patients with head/neck cancer. METHODS In this retrospective study, 39 patients with primary head/neck cancer who underwent Positron Emission Tomography (PET)/Computerized Tomography (CT) and image-guided lymph node biopsy were included. RESULTS Overall, 23 (59%) patients had biopsy-proven malignant cervical lymphadenopathy. Malignant lymph nodes had higher maximum standardized uptake (SUV-max) value (P<.001) and short-axis diameter (P=.015) compared to benign nodes. An SUV-max of ≥2.5 was 100% sensitive, and an SUV-max ≥5.5 was 100% specific for malignant lymphadenopathy. CONCLUSION The PET/CT SUV-max value can help with differentiation of malignant cervical lymph nodes in patients with head/neck cancer.

Lethal small bowel necrosis due to aspergillosis during acute promyelocytic leukemia induction

A 45-year-old female presented with persistent headache and generalized bruising. A complete blood count (CBC) showed mild anemia (11.5 g dL−1), thrombocytopenia (15 × 109/L), leukocytosis (43 × 109/L) with promyelocyte predominance (38.3 × 109/L), and a coagulopathy with INR 1.93, D-dimer > 20 μg mL−1 fibrinogen-equivalent units (FEU) (0.0–0.50 μg mL−1 FEU), and fibrinogen 150 mg dL−1 (200–420 mg dL−1). Peripheral blood and marrow aspirate smears demonstrated atypical promyelocytes with hypogranulation and multiple Auer rods (Fig. 1A). Myeloperoxidase cytochemical stain performed on the peripheral blood showed the atypical cells to be of myeloid origin (Fig. 1B). Bone marrow biopsy and aspiration showed cellularity of nearly 100% that was diffusely and extensively effaced by leukemic promyelocytes (Fig. 1C, D). Flow cytometry showed blasts expressing CD13, CD33, partial CD34, CD45, partial CD117, and partial cytoplasmic myeloperoxidase, consistent with a clonal myeloid neoplasm. Florescent in situ hybridization (FISH) analysis indicated that 95.5% of interphase cells demonstrated promyelocyte leukemia/retinoic acid receptor alpha (PML-RARA) fusion gene. G-banding karyotyping was reported as 46,XX,t(15;17)(q24.1;q21)[18]/46,XX[2]. Figure 1 Blood and bone marrow samples from patient at diagnosis. A: Diagnostic peripheral smear with leukemic promyelocytes with multiple Auer rods (left) and bilobed hypogranulated forms (Wright-Giemsa ×100 oil). B: Leukemic promyelocytes (right) staining ... The patient had high risk acute promyelocytic leukemia (APL), which is characterized by the morphologic predominance of leukemic promyelocytes and a t(15;17)(q22;q12) involving the retinoic acid receptor alpha gene. High-risk APL is characterized by a white blood cell count (WBC) at presentation of >10 × 109/L and is associated with a high risk of death during induction therapy and relapse. Vitamin A derivative all-trans retinoic acid (ATRA) is mainstay of APL treatment and APL today is considered to be the most curable subtype of all AMLs. The patient was started on all-trans retinoic acid (ATRA) 45 mg m−2, idarubicin 12 mg m−2 Days 1–4, and dexamethasone 10 mg IV every 6 hr for high risk acute promyelocytic leukemia (APL). Supportive care was provided for the patient’s coagulopathy with fresh frozen plasma, cryoprecipitate and platelet transfusions. Prophylactic antimicrobial therapy was initiated with acyclovir, levofloxacin, and fluconazole. On Day 6 of induction chemotherapy (D+6), the patient was noted to have developed peripheral edema and significant weight gain. Baseline radiograph of the chest demonstrated pleural effusions. These findings were thought due to mild ATRA or differentiation syndrome, which is generally characterized by fever and respiratory distress. However, it is also associated with weight gain, lower extremity edema, pleural or pericardial effusions, and episodic hypotension. The patient continued treatment with dexamethasone. On D+10, she developed neutropenic fever, with isolation of methicillin-resistant Staphylococcus aureus (MRSA) in blood cultures. Intravenous vancomycin and ceftazidime were started with resolution of her fever. The patient again became febrile on D+19. Chest-computed tomography (CT) showed bilateral small pleural effusions in the setting of a steroid taper. ATRA was discontinued due to concern for worsening ATRA syndrome and steroids were resumed. On this day, the patient developed abdominal distention and emesis. Abdominal CT demonstrated small bowel obstruction, thought to be secondary to ischemia, which was managed conservatively in the setting of her ongoing neutropenia and clinical stability. The patient’s antibiotic regimen was adjusted to metronidazole and piperacillin/tazobactam. Intravenous voriconazole replaced fluconazole on D+26. Additionally, she was started on granulocyte colony-stimulating factor. During this time, she noted double vision and color distortion. A head CT was unremarkable and brain magnetic resonance imaging (MRI) was delayed secondary to increased respiratory distress, which the patient developed in the setting of continued abdominal distention. She was intubated and repeat abdominal CT imaging showed a proximal closed loop small bowel obstruction (Fig. 2). Figure 2 Coronal reconstruction image of a contrast enhanced abdomen-pelvis CT demonstrates small bowel obstruction and ascites (*). The arrow points to the transition point. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.] ... Exploratory laparotomy was performed, with resection of a portion of proximal small bowel. At the time of surgery, the proximal jejunum was grossly necrotic. Small bowel pathology on D+32 showed transmural necrosis, with numerous invasive septated fungal organisms consistent with Aspergillus species and extensive angioinvasion by these organisms (Fig. 3A–D). Aspergillus galactomannan testing of serum was negative on D+32. Figure 3 Histopathological analysis of bowel resection specimen. A–D: Duodenal resection specimen. A: Extensive necrosis, ulceration with residual mucosa (hematoxylin and eosin ×10). B: Angioinvasion by fungal forms (hematoxylin and eosin ×40). ... Bone marrow biopsy on D+33 showed that she was in hematologic CR, and FISH was negative for PML-RARA fusion gene. ATRA was restarted. Possible seizure activity was noted, and a lumbar puncture (LP) and brain MRI were performed. MRI showed subarachnoid hemorrhage in the right occipital and parietal lobes. LP opening pressure was 38 cm H2O, with fluid analysis consistent with hemorrhage, glucose of 53 mg dL−1, and protein of 246 mg dL−1. The patient remained on vancomycin, piperacilin-tazobactam, and voriconazole. Serum and cerebrospinal fluid (CSF) galactomannan antigen by enzyme-linked immunoassay were both negative. On D+37, bronchoscopy showed clear airways without alveolar hemorrhage. Bronchoalveolar lavage cultures and cytology revealed no evidence of mold organisms. Candida glabrata was isolated in culture and was shown to be resistant to all tested azole drugs. Transesophageal echocardiogram was unremarkable for vegetation or shunt. ATRA was discontinued. The patient’s course was further complicated by ventilator dependence, abdominal abscess requiring drainage, continued transfusions for ongoing coagulopathy, oliguria, and probable sepsis while on antibacterial antimicrobial therapy. On D+45, an esophagogastroduodenoscopy showed a large bleeding duodenal vascular mass. On D+51, a gastrointestinal (GI) tract perforation was suspected when nasogastric tube feeds began draining through the patient’s abdominal abscess drain. A CT of the abdomen and pelvis showed a phlegmon in the left hemiabdomen containing small bowel loops and hematoma in the right hemiabdomen (Fig. 4A) as well as small bowel loops with wall thickening (Fig. 4B). Exploratory laparotomy was remarkable for necrotic bowel involving multiple loops of small bowel with transmural necrosis and perforation. The patient was transitioned to comfort cares and died on D+52. Figure 4 A: Coronal reconstructed image of a contrast enhanced abdomen and pelvis CT demonstrates a phlegmon (arrow) in the left hemiabdomen containing small bowel loops and layering blood products. Hematoma (*) in the right hemiabdomen. B: Axial images demonstrate ... Limited postmortem exam without central nervous system (CNS) examination was performed. Hemorrhagic necrosis of the distal duodenum and proximal jejunum without perforation was found, but no convincing invasive fungal organisms consistent with Aspergillus species were identified at autopsy despite very liberal tissue sampling for histopathology. The lungs were remarkable only for mild pulmonary edema and congestion. No convincing leukemic promyelocytes were seen at autopsy; however, our evaluation was limited due to the fact that granulocytes have autolytic changes at autopsy.

All that bleeds is not black: susceptibility weighted imaging of intracranial hemorrhage and the effect of T1 signal

PURPOSE To determine if intracranial hemorrhages (ICH) are always hypointense on Susceptibility weighted imaging (SWI) and to determine the effect of T1-signal intensity on the appearance of ICH in SWI series. METHODS SWI and T1-signal intensities of ICH were retrospectively studied in a series of patients. SWI signal intensities were statistically correlated with T1-signal intensities. RESULTS In a series of 57 MRI scans from 40 patients, ICH was hypointense in 19, mixed-intensity in 21, and hyperintense in 17. Hyperintensity of ICH on SWI was significantly associated with increased T1 signal (P<.001). CONCLUSION ICH can have a varied appearance on SWI.

Bosma arhinia microphthalmia syndrome: Clinical report and review of the literature.

Bosma arhinia microphthalmia syndrome (Bosma syndrome)(OMIM 603457) is a congenital condition characterized by microphthalmia with coloboma, arhinia and endocrine findings in the setting of normal intelligence and brain structure. This condition is quite rare with fewer than 50 case reports and series. Although pathogenesis is presumed to be genetic, the cause remains unknown. We report an individual with Bosma syndrome who had bilateral colobomatous microphthalmia, arhinia, high arched palate, mild ear malformations, and hypogonadotropic hypogonadism requiring growth hormone treatment in childhood, and normal intelligence. Clinical evaluation was significant for a geometrically abnormal aorta with effacement of the sinotubular ridge, a finding not previously reported in this condition. An MRI revealed absent olfactory bulbs. Suggested criteria for diagnosis of Bosma should include arhinia, hypoplastic maxilla, normal cognition, and hypogonadotropic hypogonadism in males.

Spontaneous complete resolution of pneumomediastinum and pneumatosis intestinalis caused by acute GVHD

A 31-year-old male with relapsed Hodgkin lymphoma (nodular sclerosing) after autologous haemopoietic stem cell transplantation (HCT) received allogeneic (allo) HCT from an human leukocyte antigen (HLA) identical sibling donor for consolidation in second complete remission. He presented with acute diarrhea at day 134, and was diagnosed with gut grade II acute graft-versus-host disease (aGVHD) proven by histopathological examination of a rectal biopsy. Steroids initially controlled the diarrhea, however, gut aGVHD flared at day 1100 while steroids were Image 1. CT images show air tracking in the anterior mediastinum, along the bowel wall and sub diaphragmatic region. Follow up CT images demonstrate complete resolution of the findings.

Diabetes insipidus in myelodysplastic syndrome: what we learnt from a case regarding its diagnosis, pathophysiology and management

Cull et al. recently discussed central diabetes insipidus (DI), in particular regarding its molecular and genetic abnormalities in patients with acute myelogenous leukemia (AML) [1]. We would like ...

Human Herpesvirus 6 is Associated with Status Epilepticus and Hyponatremia after Umbilical Cord Blood Transplantation

This article describes a case involving a 65-year-old man who experienced nonconvulsive status epilepticus after undergoing an allogenic hematopoietic cell transplantation to treat chronic lymphocytic leukemia. The authors describe the diagnostic work-up and treatment of human herpesvirus 6 infection, and discuss other potential causes of nonconvulsive status epilepticus in similar scenarios.