Treatment of an obstructive, recurrent, syncytial myoepithelioma of the trachea with tracheal resection and reconstruction.

Myoepithelioma is a rare occurrence in the trachea and respiratory tract with only 11 cases reported in the literature. We present a case report of a 10-year-old female who was found to have an anterior tracheal mass causing near total obstruction of the airway on bronchoscopy. Characteristics of the mass were consistent with syncytial myoepithelioma. The patient experienced multiple recurrences requiring tracheal resection with end-to-end reanastomosis. To date there have not been any reported cases of myoepithelioma of the trachea in a child and no reports of syncytial myoepithelioma in the trachea or respiratory tract.

A Pediatric Patient With an Orbital Respiratory Epithelial Cyst.

Respiratory epithelial cysts are rare orbital cysts that can arise secondary to choristomatous rests of respiratory epithelium. Approximately 15 congenital cases have been described in the literature, making it a rare disease entity. We present a case of a 14-month-old Middle Eastern male with a right infraorbital respiratory epithelial cyst. Magnetic resonance imaging of the brain and orbits revealed a right infraorbital cyst hyperintense on T1-weighted images and followed fluid density on T2-weighted images. This cyst was noted to displace the globe superiorly and inferior rectus muscle laterally. This cyst was excised using a transconjunctival approach. Histologically, the cyst wall was lined by ciliated columnar cells with interspersed mucus-containing cells and ciliated transitional epithelium was present, establishing the diagnosis of respiratory epithelial cyst. To our knowledge, this is the youngest patient with a respiratory epithelial cyst of the orbit reported in the literature.

Brainstem tegmental necrosis and olivary hypoplasia: raising awareness of a rare neuropathologic correlate of congenital apnea.

This case study describes an instance of death in an early term female newborn with congenital apnea in the clinical setting of multiple congenital anomalies (retrognathia, posteriorly rotated ears, camptodactyly, and arthrogryposis) and prenatal history of polyhydramnios. Postmortem neuropathologic findings were significant for tegmental necrosis in the caudal pons and medulla characterized by a coalescence of microcalcifications accompanied by neuronal loss, axonal spheroids, gliosis, and a concomitant hypoplasia of the inferior olives. This report raises awareness of the rare lethal entity of brainstem tegmental necrosis and olivary hypoplasia and its nosological relationship to the Möbius syndrome in the context of differential diagnosis of congenital apnea owing to central respiratory dysfunction.

Childhood primary angiitis of the central nervous system with metachronous hemorrhagic infarcts: a postmortem study with clinicopathologic correlation.

This neuropathologic case study illustrates the discovery of metachronous hemorrhagic infarcts insinuating round mass-like lesions by magnetic resonance imaging in the setting of childhood primary angiitis of the central nervous system (cPACNS) raising diagnostic awareness of this unusual presentation in a clinical and neuroimaging context. The report underscores the importance of recurrent vasculitis-induced ischemic brain damage as a pathologic correlate of relapsing cPACNS and offers a critical reappraisal of common imitators as well as a clinicopathologic approach to differential diagnosis. Attention is drawn to the caveat that although magnetic resonance imaging findings at initial presentation may not be typical for stroke, they later exhibit attributes of cerebral infarction at both the subacute and chronic stages. A pattern of cPACNS characterized predominantly by multiple petechial-like cortical hemorrhages with pathologic features of hemorrhagic infarcts is recognized. The present study lends credence to the practice of a rigorous autopsy-based approach aimed at a better understanding of the anatomic pathology and biology of cPACNS and at facilitating prospective neuroimaging and biopsy-based surgical pathology correlations, ultimately enhancing diagnostic accuracy in clinical settings. Although PACNS is, by definition, a diagnosis of exclusion, it should be considered from the outset in the differential diagnosis of ischemic stroke or hemorrhagic stroke or of unusual and relapsing intra-axial mass-like CNS lesions in children, necessitating appropriate pathologic evaluation of brain biopsy specimens.

Chemotherapy-related leiomyopathy: a suggested morphological explanation for the intestinal dysmotility affecting patients treated with anthracyclines.

Anthracycline, used in oncological chemotherapy, has one well-known side effect: cardiotoxicity. Another is abnormal intestinal motility such as constipation and ileus, the pathogenesis of which, to our knowledge, has not been morphologically investigated. We conducted a study in search of morphological evidence that might shed some light on the pathogenesis of the motility dysfunction. Autopsies performed between 2002 and 2007 were reviewed to select cases of children who had received anthracycline therapy for various neoplasms. The seven patients found had leukemias, lymphomas, or renal solid tumors. They all suffered from constipation or intestinal dysmotility, and no case of anthracyclin-treated neoplasia without the side effect was found in the files. Tissue samples from the heart, gastrointestinal tract, uterus, urinary bladder, and skeletal muscles were examined by light and electron microscopy. As described by others, the myocardium of all anthracycline-treated patients showed loss of myofilaments, fibrosis, mitochondrial proliferation, and pools of accumulated Z-band material. In the gastrointestinal tract and other smooth muscle-endowed organs such as muscular blood vessels, bladder and uterus, the muscularis displayed hyalinization and disorganization, including loss of myofilaments and moderate-severe fibrosis. This study illustrates changes in the smooth muscle, and that of the gastrointestinal tracts and their vessels in particular, in patients treated with anthracycline, who had experienced motility dysfunction associated with their chemotherapy, suggesting that, in addition to the heart, anthracycline may also damage smooth muscle fibers and thus be instrumental in the pathogenesis of the side effects.

Appendiceal goblet cell metaplasia and benign obstructive mucus retention in children: report of eight cases and review of the literature.

We report the clinical-pathologic study of 8 cases of pediatric benign, postobstructive, appendiceal mucus retention in patients 3-15 years of age. The appendices showed very limited acute inflammation. Their most significant change consisted of minimal to mild dilatation of the distal lumen, which was filled with mucus and lined by an epithelium showing often dramatic increase in mucus cells devoid of atypia and without any of the villous proliferation reported in instances of "mucosal hyperplasia" observed in adult patients. In all 8 cases, there was seepage of mucus through the appendiceal wall into the surrounding tissue, eliciting a macrophagic reaction. Proximal to the zones of mucus retention, the lumen of 2 of the appendices was obstructed by a fecalith, and 2 exhibited fibrous obliteration of the lumen at that site. We believe the condition results from obstruction and focal inflammation, which isolate the distal portion of the appendix and elicit goblet cell metaplasia, the secretion of which is followed by luminal distention, eventual rupture, and seepage of mucus within the wall and the mesoappendix. The clinical and radiologic picture of this condition may be mistaken for that of an acute appendicitis complicated by rupture and abscess formation. The report provides an opportunity to describe and characterize "appendiceal mucosal goblet cell metaplasia," a seldom mentioned entity associated with benign appendiceal obstructive mucus retention in children.

Recognition of Crohn disease on incidental gastric biopsy in childhood.

The role of gastric biopsy in the diagnosis of Crohn disease (CD) in the pediatric population has not been well described. We assessed the use of gastric biopsies in the diagnosis of CD using specific histopathologic parameters: granulomata, focal gland injury with neutrophils (glandulitis or glandular abscesses), and/or focal concomitant eosinophilic infiltrates. Multiple (438) consecutive pediatric biopsies with inflammation spanning a 5-year period were identified from archival material in patients ages 2 months to 16 years. A total of 56 CD cases were confirmed using colon biopsies and clinical and radiologic data as the gold standards of diagnosis. Review of hematoxylin and eosin (H&E) slides and Diff-Quik stained slides (negative for Helicobacter pylori) isolated 53 cases which suggested CD on gastric biopsy: 20 cases with granulomata, 14 cases with focal glandulitis and glandular abscesses, and 19 cases of focal glandulitis/glandular abscesses with eosinophilic infiltrates. Seventy-seven percent (43/56) were correctly identified as patients with CD. Twenty-three percent (13/56) of CD cases were not identified primarily because of concurrent H. pylori infection identified on Diff-Quik stain with a superimposed nonspecific diffuse gastritis. The use of Diff-Quik stain to identify H. pylori cases after all other factors are considered was significant (P = 0.0145); a negative stain, combined with the identified histopathologic features indicative of CD, significantly increased the accuracy of CD diagnosis. CD was mimicked by other gastric granulomatous diseases (actinomyces, 1 case; chronic granulomatous disease of childhood, 1 case). Gastric biopsy can be used to identify or support the diagnosis of CD in children in the appropriate clinicopathologic setting.