| Pulmonary manifestations of pheochromocytoma are infrequent and are not well documented. A medline search in the English language revealed no cases of endobronchial involvement from a pheochromocytoma. We report a case of endobronchial metastases in a 37-year-old woman known to have a recurrent extra-adrenal pheochromocytoma. She presented with symptoms of wheezing and a nonproductive cough for 8 months and was being treated for asthma. A flexible bronchoscopy with endobronchial biopsy established the diagnosis. The patient underwent a Nd-YAG laser photoresection (LPR) to ablate the tumor, which was followed by placement of a Wallstent (Pfizer Medical technology Group; Rutherford, NJ). She remains well 18 months later, having required multiple palliative LPRs. To our knowledge, this is the first reported case of endobronchial pheochromocytoma. The pulmonary manifestations of this rare disease and their management are reviewed. ( info) |
| A paraganglioma of cauda equina region is extremely rare and except for secreting tumour, the pre-operative diagnosis of paraganglioma is very difficult. Two cases of non-functional paragangliomas of the cauda equina region are reported, one was attached to the filum terminale and the other to a rootlet looking very much like a vascular neurinoma. Both were successfully removed by surgery. An extensive review of the literature permits one to find 77 other cases. The clinical, radiological, pathological (ultrastructural and immunohistochemical) features and surgical findings of all theses cases are discussed. Surgery remains the treatment of choice. No effect of radiotherapy on recurrence prevention has ever been demonstrated. ( info) |
3/534. Bone metastases from a paraganglioma. A review of five cases. Paragangliomas are infrequent, usually benign tumors developed from neuroectoderm cells. The neck is the most common location, although some cases arise within the abdominal cavity, usually in the retroperitoneal space. We report five cases with bone metastases. In three patients, convincing evidence was obtained that the primary was in the retroperitoneal space. Clinical manifestations of metastatic bone disease occurred up to 17 years after the diagnosis of paraganglioma. Useful data were obtained from plain radiographs, magnetic resonance imaging, serum and urine catecholamine assays, and above all meta 123iodobenzylguanidine scintigraphy. Histologic and immunohistochemical studies of the lesion yielded the definite diagnosis. Surgery and radiation therapy are the two mainstays of therapy. Although rare, metastatic forms of paraganglioma should be borne in mind. This diagnosis should be entertained in patients with bone lesions and recent-onset arterial hypertension, irrespective of whether they report a history of surgery for a tumor, and even if this tumor was removed many years earlier and labeled benign. ( info) |
4/534. KTP laser assisted excision of glomus tympanicum. A 39-year-old female with a two-year history of mild hearing loss and discomfort on air flight descent was found to have a pulsatile mass behind an intact tympanic membrane. A suspected diagnosis of glomus tympanicum was confirmed by computed tomography (CT) scan imaging. The lesion filled the mesotympanum and hypotympanum but the jugular bony plate was intact, confirming the tympanic site of the lesion. This very vascular tumour was exposed by a tympanomeatal flap and the KTP laser used to shrink and coagulate the tumour progressively with minimal haemorrhage and blood loss. Complete excision of the lesion was achieved without the need for bony removal, and with minimal blood loss. The use of the KTP laser to coagulate this vascular lesion allowed safe removal of the tumour and avoided the need for extended facial recess or hypotympanotomy surgery. ( info) |
5/534. Intrasellar paraganglioma presenting as nonfunctioning pituitary adenoma. Paragangliomas arising in the sellar region are rare. We report a case of intrasellar paraganglioma of a 54-year-old man who presented with gradually decreasing visual acuity. physical examination revealed bitemporal hemianopsia with no apparent signs of endocrinologic dysfunction. magnetic resonance imaging revealed a large sellar mass believed to be a nonfunctioning pituitary adenoma. The tumor was removed transsphenoidally and submitted for histologic examination. The morphologic features, based on histologic, immunocytochemical, and electron microscopic analyses, were consistent with the diagnosis of paraganglioma. The diagnostic morphologic features and the immunocytochemical profile of the tumor are reported. ( info) |
6/534. The incidental diagnosis. incidental findings transcend medical practice. Two cases are discussed in which significant conditions were discovered during clinical examination for an unrelated disorder. The first case presents a disease reported for the first time in a male. Recent literature has alluded to missed diagnoses found only at autopsies. With cost constraint as a national theme, the appropriate use of technology presents a challenge to the physician. The cases discussed raise questions about the process of diagnosis. Both situations bring to light the limitations of clinical investigation early in the pathologic process. ( info) |
7/534. Laryngeal paraganglioma in a five-year-old child--the youngest case ever recorded. A paraganglioma is a neuroendocrine neoplasm that originates from the paraganglion cells of the parasympathetic system. The average age of presentation is in the fifth decade. We report a case of laryngeal paraganglioma in a five-year-old child, the youngest case ever recorded. The features of paraganglioma which differentiate it from other tumours are also discussed. ( info) |
8/534. Visualisation of a paraganglioma by technetium-99m-sestamibi scintigraphy. A 68-yr-old woman presented to our observation with multinodular goiter and a contiguous right laterocervical mass. In spite of ultrasound, technetium and iodine scan, CT and fine-needle biopsy, the precise origin of the mass remained uncertain. On additional multi-phase sestamibi scan, the neck region showed an early high uptake rapidly decreasing over time in the laterocervical mass, and a persistent inhomogeneous distribution in the thyroid gland. This behavior suggested that the laterocervical mass could derive from an anatomical structure other than the thyroid. Surgical exploration established the extrathyroid nature of the laterocervical mass and the histological examination confirmed that it was a typical paraganglioma. This finding is in keeping with a recent report of positive sestamibi uptake in a cervical paraganglioma, although our case showed a more rapid kinetic. This tumor should be therefore taken into consideration in the differential interpretation of focal sestamibi uptake. ( info) |
| A 56-year-old female presented with a paraganglioma in the left anterior cranial fossa who manifesting as persistent headache. Computed tomography and magnetic resonance imaging showed a solid, enhanced tumor with a cystic component located medially. The tumor was attached to the left frontal base and the sphenoid ridge. angiography demonstrated a hypervascular tumor fed mainly by the left middle meningeal artery at the left sphenoid ridge. The preoperative diagnosis was meningioma of the left frontal base. The tumor was totally resected via a left frontotemporal craniotomy. Histological examination revealed the characteristic cellular arrangement of paraganglioma generally designated as the "Zellbaren pattern" on light microscopy. Only 10 patients with supratentorial paraganglioma have been reported, seven located in the parasellar area. The origin of the present tumor may have been the paraganglionic cells which strayed along the middle meningeal artery at differentiation. ( info) |
| The MR findings of a case with paraganglioma of the cauda equina are presented. T2-weighted images showed the tumor to have a hyposignal rim and serpiginous flow voids, suggesting vessels capping the tumor. Contrast-enhanced MR images highlighted the tumor and tumor vessels more clearly. knowledge of MR findings in this unusual disorder may aid in diagnosis. ( info) |