SUMMARY: Although a relatively rare neoplasm, primary carcinoid tumor has an unusual propensity to metastasize to the orbits. Within the orbit, metastatic EOM lesions have been described in scattered reports in the ophthalmology literature but have received little to no attention in the radiology literature. After a retrospective review, we identified CT and MR imaging studies of 7 patients with carcinoid tumor metastatic to the EOM. Our findings suggest that in patients with known carcinoid tumor, well-defined, round, or fusiform masses of the EOM should strongly suggest metastatic involvement. Our series suggests that bilateral lesions may occur and that any EOM can be involved. Knowledge of this pattern of metastatic disease may spare biopsies in some patients, and with current orbit-sparing therapy for patients with localized orbital disease, early and accurate diagnosis can significantly improve patient outcomes.ABBREVIATIONS: CN VI ϭ cranial nerve VI; EOM ϭ extraocular muscle; IO ϭ inferior oblique; IR ϭ inferior rectus; LR ϭ lateral rectus; MR ϭ medial rectus; MRI ϭ MR imaging; N/A ϭ not applicable; SO ϭ superior oblique; SR ϭ superior rectus C arcinoid tumors are rare neuroendocrine neoplasms derived from enterochromaffin cells, which are found primarily in the gastrointestinal tract and bronchial tree.1,2 Liver metastases are the classic presentation of distant disease, which can lead to carcinoid syndrome (flushing, diarrhea, and wheezing) and right-sided valvular heart disease. However as treatment options improve and survival increases, new metastatic patterns have been increasing in frequency.3 Although rare, metastatic carcinoid to the extraocular muscles has been relatively well described in both retrospective case reports and clinical series in the ophthalmology literature. [4][5][6][7][8][9][10][11][12][13] However, by our review of the radiology literature, there is a single dedicated case report detailing the imaging findings for this entity.14 Even within the ophthalmology literature, only a handful of reports focus exclusively on metastases involving the EOMs. 4,8,15 We present the first series detailing the CT and MR imaging findings of carcinoid tumor metastases to the EOMs. Materials and MethodsA waiver of informed consent was obtained from our institutional review board, and in a manner consistent with Health Insurance Portability and Accountability Act regulations, we retrospectively collected clinical data and CT and MR images available from our institution in patients with known carcinoid tumor. In addition, via a survey of head and neck radiologists from collaborating institutions, additional cases were collected. All imaging analyzed for this study was performed from January 2000 through October of 2010.We found a total of 7 patients with known metastatic disease to the EOMs. The primary inclusion criterion was the presence of EOM lesions in patients with known metastatic carcinoid tumor, though the presence of non-EOM intraorbital disease was not considered an exclusion criterion. ...
Paragangliomas are rare neuroendocrine tumors that result from the abnormal migration of neural crest progenitor cells, or paraganglia, during embryonic development. Paraganglia in the head and neck migrate along a branchial mesoderm; therefore, head and neck paragangliomas may occur anywhere along the branchiomeric distribution. Head and neck paragangliomas demonstrate a number of characteristic features, such as common anatomic locations, symptomatology, associated genetic mutations, and appearance on multimodal imaging. Understanding these important attributes can allow for a prompt and accurate diagnosis. This article provides a pictorial review of the common imaging features of head and neck paragangliomas.Learning Objective: Describe the multimodal imaging appearance of head and neck paragangliomas and their common anatomic locations.
Introduction/Objective Rapidly progressive glomerulonephritis is characterized by sudden decline in renal function and glomerular crescents. Crescentic glomerulonephritis develops along three pathways: glomerular basement membrane antibody in situ deposition , circulating immune complex glomerular deposition, or pauci-immune (typically ANCA-associated). For the latter, histopathologic diagnosis is more frequently obtained in adults than in children. Therefore, tissue examination opportunities in infants and neonates is exceptionally rare. Methods/Case Report Here we report the autopsy findings from a 4-week-old infant who was part of dichorionic, diamniotic twin pregnancy and born prematurely at 34 weeks to a 30-year-old G6P4 mother. The infant initially presented with labored breathing and poor oral intake, but subsequently developed Enterobactor meningoencephalitis and sepsis, leading to extensive strokes andstatus epilepticus. An infectious source was not identified clinically, and evaluation for an underlying immunodeficiency was indeterminant. The mother had no known autoimmune diseases or antenatal infections. At autopsy, kidney histology showed focal fibrinoid necrosis and/or cellular and fibrocellular crescents (~5%), especially mid to deep cortex. Intracapillary hypercellularity was minimal to absent. Mesangial matrix was not expanded. The interstitium contained patchy inflammatory infiltrates (mononuclear > polymorphonuclear). Direct immunofluorescence (IF) on pronase-treated paraffin sections did not show glomerular deposition of IgG, IgA, or IgM. Electron microscopy (EM) did not show electron dense deposits despite autolysis. Results (if a Case Study enter NA) NA. Conclusion The absence of immune complex deposition by IF and dense deposits by EM favors the rare diagnosis of pauci-immune glomerulonephritis in this case of neonatal crescentic glomerulonephritis associated with Enterobactor meningoencephalitis and sepsis, with no associated maternal autoimmune disease or antenatal infections.
A 68 year-old Caucasian male presented with weight loss, fatigue and weakness. Diagnostic studies including bone marrow and liver biopsies, cardiac catheterization, colonoscopy and pleural tap of chylothorax were reported with negative results. A remote history of colon cancer with radiation and chemotherapy was noted. Clinical concern over possible amyloidosis precipitated a renal biopsy.Needle cores of renal cortex were received in 10% buffered Formalin, 3% buffered glutaraldehyde or Michel's buffer and processed using routine methodology for brightfield, electron and immunofluorescent microscopy, respectively. Formalin-fixed tissue was embedded in paraffin, sectioned 3-um in thickness and examined by brightfield microscopy after application of routine and special histological stains. Glomeruli were normocellular by H&E stain with no specific alterations in extracellular matrix using PAS and silver stains. PAS-positive, Gram-negative rods were seen coating podocytes in Bowman's space and along epithelial cells of tubules. Thioflavin-T stain was negative for amyloid under fluorescent light. Tissue cores submitted for direct immunofluorescence lacked glomeruli upon frozen sectioning; therefore, immunolabeling was not performed.One-micron thick sections of glutaraldehyde-fixed kidney allowed for the selection of one glomerulus for examination by transmission electron microscopy. Ultrastructural examination showed numerous rod shaped or bacilliform structures in Bowman's space between and adjacent to visceral and parietal epithelial cells. Rods were seen within lumina of renal tubules. Organisms were identified within parietal and tubular epithelial cells and a few rods were found within podocytes. Isolated electron dense immune-type deposits were detected in mesangial matrix and along the subepithelial aspect of glomerular capillary loop basement membranes, the latter forming humps. Amyloid fibrils were not identified. T. whippelii was identified by PCR amplification using tissue retrieved from the paraffin block.
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