Section on Pathology. (Abstracts of Scientific Posters).

PATH1-D. PRIMARY RENAL CARCINOID TUMOR: AN ULTRASTRUCTURAL AND IMMUNOHISTOCHEMICAL STUDY Moses Adedeji, MD, and Steve Bigler, MD.

Primary carcinoid tumors are found mostly in the gastrointestinal tract. Primary renal carcinoid tumor is rare. Less than 50 cases have been documented in the medical literature, and some of these cases were published twice. This unusual tumor is overrepresented in patients with horseshoe-shaped kidney (20% of reported cases). The histogenesis of primary renal carcinoid tumors remains unclear, and it is still disputed. We present the case of a 47-year-old black man who underwent radical nephrectomy for a presumed renal cell carcinoma. The neoplasm was characterized at the gross, microscopic and ultrastructural levels, and a panel of immunohistochemical stains was performed. The kidney weighed 362 grams and measured 15.0 X 8.0 X 6.5 cm. The kidney was bisected to reveal a tan-pink and red mass occupying and distending the renal pelvis. The noncystic mass measured 5.0 X 4.0 X 4.0 cm, and it was well-confined within the renal and pelvicalyceal system. Microscopically, the tumor was composed of monotonous cells w ith trabecular and insular patterns, with cytoplamic expression of low molecular-weight cytokeratin, synaptophysin, and vimentin, and negative for high molecular-weight cytokeratin, carcinoembryonic antigen, S100, and glucagon. Electron microscopy revealed two types of tumor cells (cells with dark cytoplasm and cells with light cytoplasm), both containing large numbers of membrane-bound, cytoplasmic dense-core neuro-secretory granules. Carcinoid tumor of the kidney is a rare neoplasm that may present with radiographic and clinical features suggestive of urothelial carcinoma or renal cell carcinoma.

PATH2-D. NASAL GLIOMA PRESENTING AS SLEEP APNEA IN A 43-YEAR-OLD MAN: A CASE REPORT AND REVIEW OF THE LITERATURE. Zohreh Zaki, MD, Harsharan K. Singh, MD, and Marcus S. Albernaz, MD. East Carolina University Brody School of Medicine, Department of Pathology and Laboratory Medicine, Pitt County Memorial Hospital, Greenville, NC.

Nasal gliomas are rare, benign, congenital lesions that are usually diagnosed during infancy and early childhood. Sixty percent of these lesions are extranasal, 30% are intranasal, and the remaining are mixed. These lesions occur in both sexes, with a slight male predominance. Presentation during adult life is extremely rare. We report the case of a 43-year-old man who presented with sleep apnea. During work-up for his symptoms, fiberoptic nasal endoscopy and direct laryngoscopy revealed redundancy of oropharyngeal and uvulopharyngeal tissues, and a left middle meatus polyp. CT scan of the paranasal sinuses showed polypoid mucoperiosteal thickening of the left ethmoid air cells. No basal skull bone defect or intracranial lesion was identified. He underwent uvulopalatopharyngoplasty and tonsillectomy and a nasal surgery with ethmoid polyp resection. Histologic examination of the polyp revealed a nasal cerebral heterotopia (nasal glioma). His symptoms improved after surgery with no further complication. This ca se illustrates one of the few cases of nasal gliomas diagnosed in adults. In conclusion, nasal gliomas are extremely rare in adults. They usually present as intranasal mass and should be considered in differential diagnosis of nasal masses. Our case is one of the few cases of adult nasal glioma and is unique in the way of presentation. CT scan and MRI are the mandatory modalities to rule out intracranial connection before manipulation of the lesion. The treatment of choice is excision via an intranasal approach, usually without further complications.

PATH3-D. USE OF ROBOTIC TELEPATHOLOGY FOR FROZEN SECTION DIAGNOSIS. Keith J. Kaplan, MD, Jeanette B. Benedict, MD, Glenn D. Sandberg, MD, Cris P. Myers, MD, Thomas R. Bigott, BS, and Renata B. Greenspan MD. Department of Pathology, Walter Reed Army Medical Center, Department of Neuropathology Armed Forces Institute of Pathology, Washington DC, and Department of Pathology, Heidelberg Army Hospital, Heidelberg, Germany.

Telepathology is the practice of digitizing histologic or macroscopic images for transmission along telecommunication pathways for diagnosis, consultation, or continuing medical education. Previous studies have addressed static versus dynamic imaging in several specimen types, with a wide variety of systems and communication pathways. The goal of this paper was to assess the validity of a Web-based telepathology system for frozen-section consultation within the Army Medical Department (AMEDD). The system provides real-time, dynamic remote control of a robotic microscope over standard Internet connections. Oftentimes, a solo pathologist is called upon to provide diagnostic services without the support of immediate second/expert consultation during an intraoperative consultation. The use of telepathology is attractive because it provides an opportunity for pathologists to obtain immediate consultation. Telepathology systems were initially installed at the hospitals serving Fort Knox (KY), Fort Bragg (NC) & Land stuhl Regional Medical Center (Germany). 130 consecutive frozen-section cases were reviewed from throughout the AMEDD at a distance. Use of consecutive cases eliminated bias related to case complexity. Intraobserver agreement between the telepathology diagnosis and glass slide diagnosis and time to diagnosis (TTD) was observed. Specimens from 21 organ systems/sites were obtained. Indications for the frozen section were for primary diagnosis, extent of resection (margins), or intraoperative staging. Diagnostic agreement was 100% for a wide variety of specimens. Overall, TTD was less than 3 minutes using the telepathology system. No telepathology sessions were interrupted due to technical failure of the system, and no diagnoses were deferred pending evaluation of the glass slides. This study suggests that such a system will help support pathologists located at distant sites.

PATH4-D. HODGKIN'S-LIKE TRANSFORMATION IN CHRONIC LYMPHOCYTIC LEUKEMIA PRESENTING AS A LIVER MASS DIAGNOSED BY FINE-NEEDLE ASPIRATION BIOPSY Keith J. Kaplan MD, and Anthony A. Corsini MD. Department of Pathology, Walter Reed Army Medical Center, Washington, DC.

We report a case of Hodgkin's-like (HD-L) transformation in chronic lymphocytic leukemia (CLL) presenting as a liver mass diagnosed by fine-needle aspiration biopsy (FNAB). The patient is a 76-year-old woman with a 20-year history of CLL/small lymphocytic leukemia (SLL) who had undergone two cycles of fludarabine therapy. CT scan was performed due to worsening clinical symptoms. The patient was discovered to have a hypointense liver mass in the right lobe. CT-guided FNAB of the mass was performed. Biopsy revealed a lymphohistiocytic-rich proliferation with scattered, large, Hodgkin's-like cells within the liver parenchyma. These cells were focally positive for CD3O (Ki1) and Ebstein-Barr virus latent membrane protein-1 (EBVLMP-1) and negative for CD15 (Leu-M1). An association between fludarabine treatment and transformation of CLL/SLL and other non-Hodgkin's lymphomas to HD-L lymphomas has been reported. Most of these cases are diagnosed at postmortem examination, with diffuse disease involving multiple lymph nodes and involvement of soft tissue structures. EBV-LMP-1 is often associated. The overall prognosis is poor. The differential diagnosis includes large cell lymphoma. To our knowledge, there is only one other case report in the literature documenting HD-L transformation diagnosed by FNAB involving a periaortic lymph node. This is the first reported case of HD-L transformation in CLL/SLL diagnosed by FNAB in an extranodal site. FNAB has become increasingly popular in the diagnosis and management of patients with lymphoproliferative disorders. Thus, cytologists and pathologists must be award of HD-L transformation in the setting of low-grade non-Hodgkin's lymphomas, particularly following fludarabine therapy. We recommend tissue specimens be obtained for flow cytometry and molecular studies. Prognosis is affected by early recognition and detection of the transformation.

PATH5-D. MANTLE CELL LYMPHOMA WITH CENTRAL NERVOUS SYSTEM INVOLVEMENT: A CASE REPORT AND THE ROLE OF THE LABORATORY

Zohreh Zaki, MD, and H. James Williams, MD. East Carolina University/ Brody School of Medicine, Department of Pathology and Laboratory Medicine, Pitt County Memorial Hospital, Greenville, NC.

Mantle cell lymphoma (MCL) is a type of non-Hodgkin's lymphoma which arises from the mantle zone of the lymphoid follicle. It is composed of a proliferation of atypical lymphoid cells that range from typical to blastoid variants with diffuse or nodular patterns. Immunophenotypically, the cells are monoclonal B-lymphocytes with CD5 coexpression. Genetically, this lymphoma is characterized by t(11;14) (q13;q32) with bcl-l rearrangement and overexpression of cyclin D1. Extranodal involvement is common and occurs in advanced stages of the disease, with bone marrow, gastrointestinal tract, and spleen being the most common sites. Central nervous system (CNS) involvement is rare, and only a few cases have been reported. We present a 69-year-old man who was found to have diffuse lymphadenopathy, hepatosplenomegaly, and peripheral blood (PB) leukocytosis with numerous blasts. A bone marrow biopsy was performed and, based on morphologic, immunophenotypic and cytogenetic studies, a diagnosis of MCL was made. He underwen t chemotherapy, and his PB was cleared. Ten days after chemotherapy, he developed generalized weakness and paresthesia. A lumbar puncture was performed and revealed atypical lymphocytes. Flow cytometry (FC) analysis showed immunophenotype consistent with MCL. He received intrathecal chemotherapy with subsequent clearing of CSF. CNS involvement is rare in low-grade lymphomas, and only a few cases of MCL with early CNS involvement have been reported. When CNS involvement occurs, it is usually late in the course of the disease and is associated with a poor prognosis. FC analysis plays an important role in distinguishing reactive lymphocytes from malignant cells in CSF, since cytology can not provide information about pattern of growth and effacement of the architecture. FC is a rapid and sensitive method for detection of malignant lymphoid cells in CSF.