Jugular foramen: microscopic anatomic features and implications for neural preservation with reference to glomus tumors involving the temporal bone.

OBJECTIVE: Our goals were to study the normal histological features of the jugular foramen, compare them with the histopathological features of glomus tumors involving the temporal bone, and thus provide insight into the surgical management of these tumors with respect to cranial nerve function. METHODS: Ten jugular foramen blocks were obtained from five human cadavers after removal of the brain. Microscopic studies of these blocks were performed, with particular attention to fibrous or bony compartmentalization of the jugular foramen, the relationships of the caudal cranial nerves to the jugular bulb/jugular vein and internal carotid artery, and the fascicular structures of the nerves. In addition, we studied the histopathological features of 11 glomus tumors involving the temporal bone (10 patients), with respect to nerve invasion, associated fibrosis, and carotid artery adventitial invasion. RESULTS: A dural septum separating the IXth cranial nerve from the fascicles of Cranial Nerves X and XI, at the intracranial opening, was noted. Only two specimens, however, had a septum (one bony and one fibrous) producing internal compartmentalization of the jugular foramen. The cranial nerves remained fasciculated within the foramen, with the vagus nerve containing multiple fascicles and the glossopharyngeal and accessory nerves containing one and two fascicles, respectively. All of these nerve fascicles lay medial to the superior jugular bulb, with the IXth cranial nerve located anteriorly and the XIth cranial nerve posteriorly. All nerve fascicles had separate connective tissue sheaths. A dense connective tissue sheath was always present between the IXth cranial nerve and the internal carotid artery, at the level of the carotid canal. The inferior petrosal sinus was present between the IXth and Xth cranial nerves, as single or multiple venous channels. The glomus tumors infiltrated between the cranial nerve fascicles and inside the perineurium. They also produced reactive fibrosis. In one patient, in whom the internal carotid artery was also excised, the tumor invaded the adventitia. CONCLUSION: Within the jugular foramen, the cranial nerves lie anteromedial to the jugular bulb and maintain a multifascicular histoarchitecture (particularly the Xth cranial nerve). Glomus tumors of the temporal bone can invade the cranial nerve fascicles, and infiltration of these nerves can occur despite normal function. In these situations, total resection may not be possible without sacrifice of these nerves.

Absence of peritumoral Crooke's change is associated with recurrence in surgically treated Cushing's disease.

BACKGROUND: Pituitary surgery is the standard treatment for Cushing's disease but is complicated by a recurrence rate that ranges from 5.9 to 27%. Whereas some recurrences may be due to technical or anatomical factors resulting in subtotal resection of adenoma, clinical relapse after total tumor resection is a well-documented occurrence. The factors leading to such recurrences are unknown. METHODS: With the hypothesis that the pathology of the nontumoral adenohypophysis is important in predicting relapse, we undertook a study to determine if the absence of Crooke's change (CC), thought to be an indicator of nontumoral corticotroph inhibition, was associated with unexpected clinical recurrence. Twenty-one patients with Cushing's disease, with gross total resection of intrasellar corticotroph microadenoma, were reviewed independently by 2 neuropathologists for the presence of CC in adjacent adenohypophysis. All tumors were stained with H&E, PAS/orange-G and immunohistochemistry for ACTH. Clinical relapse was determined by chart reviews and defined as serum ACTH > 15 pg/ml, clinically Cushingoid, and/or radiographic evidence of recurrent tumor. RESULTS: Seven of 21 patients recurred; 3 did not have CC in their initial resection specimen. All 3 of these patients had unexpected recurrences at 6 to 48 months post-op. Two patients with CC recurred at one year follow-up, 1 after 4 years and 1 after 5 years. All specimens from patients with long-term cure (follow-up from 9-72 months) contained CC. In this study, the absence of CC in peritumoral adenohypophysis was associated with unexpected recurrence of Cushing's disease (p = 0.0214). CONCLUSIONS: We conclude that absence of CC in peritumoral adenohypophysis may be of some assistance in predicting recurrence of Cushing's disease after adequate resection of intrasellar microadenoma.

Meningiomas involving the cavernous sinus: histological factors affecting the degree of resection.

Despite advances in the surgical treatment of meningiomas located at the skull base, surgery for meningiomas involving the cavernous sinus remains controversial. The controversy centers on whether complete resection of such a meningioma is possible while preserving cranial nerve function. To evaluate this question, the authors examined six patients with benign meningiomas involving the cavernous sinus. The pathological features of these tumors were evaluated and compared with the normal histoarchitecture of the cavernous sinus. The tendency of these tumors to be infiltrative is evident and this seems to occur along connective tissue planes within the cavernous sinus. This invasiveness can be explained by the peculiar structure of this region. The trigeminal nerve and ganglion seem to be particularly prone to invasion; this does not correlate with the degree of preoperative impairment of nerve function. Internal carotid artery invasion occurs frequently and can be seen even when there is no narrowing of the artery on arteriography. The pituitary gland can also be invaded by the tumor, which penetrates the thin dural barrier.

The distribution of Lewy bodies in pure autonomic failure: autopsy findings and review of the literature.

Pure autonomic failure (PAF; also known as idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome) is an uncommon sporadic disorder, characterized by autonomic failure without other neurological deficits and histopathologically by cell loss in intermediolateral columns and sympathetic ganglia. Few postmortem studies of patients with PAF have been reported in the literature, and none have demonstrated Lewy bodies in distal axons, although this has been described as a feature in Parkinson's disease with autonomic failure. We report a patient with PAF who had orthostatic hypotension and urinary symptoms for 15 years prior to death at the age of 63 years. Postmortem findings included typical and atypical Lewy bodies in the substantia nigra, locus ceruleus, substantia innominata, and sympathetic ganglia, as well as in autonomic axons in the epicardial fat, autonomic nerve fascicles in periadrenal adipose tissue, and autonomic nerves in the muscularis of the urinary bladder. Sites of autonomic nerve involvement correlated with clinical symptomatology, and thus were a valuable observation in the complete autopsy. Systemic autopsy results should be reviewed carefully in patients with PAF, as Lewy bodies in this disease may be seen in distal axons at a great length from their primary cell bodies.

Craniopharyngioma arising de novo in middle age. Case report.

The authors report the case of a 55-year-old woman who developed a symptomatic craniopharyngioma within 2 years of obtaining a normal magnetic resonance image of her brain. Craniopharyngiomas are histologically benign tumors. They are thought to arise from embryonic remnants of Rathke's pouch and sac and to manifest themselves clinically after a steady growth that commences in fetal life. To the authors' knowledge, this is the first report that documents a tumor arising de novo in the sixth decade of life. This report appears to challenge the concept of the origin and natural history of craniopharyngiomas.

Posttransplant lymphoproliferative disease presenting as sudden respiratory arrest in a three-year-old child.

Posttransplant lymphoproliferative disease (PTLD) is an occasional complication of pediatric organ transplantation that, heretofore, has not been associated with airway obstruction. We report the first documented case of PTLD associated with complete airway obstruction resulting in sudden respiratory arrest and death in a 3-year-old child. This is contrasted to a subsequent case of PTLD wherein heightened clinical suspicion and prompt tonsillectomy resulted in a definitive diagnosis and improved outcome. The early clinical hallmarks of PTLD are a mononucleosis-like syndrome, tonsillar enlargement, and positive Epstein-Barr virus seroconversion. The potential for a fatal outcome of PTLD involving Waldeyer's ring components warrants early recognition and aggressive treatment.

Fine needle aspiration of a renal cell carcinoma with eosinophilic globules. A case report.

Fine needle aspiration (FNA) of a renal cell carcinoma with hyaline eosinophilic globules is presented. The cytology of the tumor cells was characterized by intracytoplasmic, dense, well-circumscribed globules that had a bright magenta appearance on Diff-Quik staining. Tissue sections demonstrated the globules to be located predominantly within the cytoplasm of the clear cells of the carcinoma. Immunohistochemical and ultrastructural evaluation is discussed, as is the significance of this finding.

A Bangladeshi family with three sisters 'Bombay' or Oh phenotype.

Three sisters in a same family (MIAH FAMILY) are of 'Bombay' phenotype. These being the first known female examples of 'Bombay' blood group have been detected in Bangladesh. As predicted by current theory their red cells are Le(a+b-) and their saliva do not contain any of the antigens A, B and H except Lea substance. Family studies showed that individuals with 'Bombay' or Oh phenotype may have A or B gene which are not expressed. This very particular type of blood is one of the rarest in any other parts of world except in India. Due to the presence of anti-H antibody in the plasma of Oh phenotype, when considering such patients for transfusion only blood of identical Bombay type can be safely transfused.