Cushing's syndrome due to medullary thyroid carcinoma: diagnosis by proopiomelanocortin messenger ribonucleic acid in situ hybridization.

Medullary thyroid carcinoma (MTC) rarely causes ectopic ACTH syndrome. We describe a 38-yr-old man with renal stones who had a 5-cm MTC removed in 1992. He was RET-protooncogene positive (codon 618). Serum calcitonin was 1597 pg/ml postoperatively. In 1996 he had rib fractures, bruising, weakness, and three to four stools per day. Laboratory studies revealed an elevated 24-h urine-free cortisol (780 micro g/d), epinephrine (66 micro g/d), and calcium (558 mg/d). Baseline serum cortisol was 23.9 micro g/dl and decreased to 12.9 and 4.5 micro g/dl after 2 mg and 8 mg dexamethasone suppression, respectively. Plasma ACTH was 170 pg/ml and decreased to 75 and 24 pg/ml after dexamethasone. Bone density t-score was -4.3 (trochanter). Computed tomography scans showed multiple cervical nodes and 2-cm right adrenal nodule. Magnetic resonance imaging (MRI) scan showed a prominent, homogeneous pituitary; the adrenal MRI scan was not typical for a pheochromocytoma. Serum CRH was less than 6.6 pg/ml. Bilateral adrenalectomy revealed two adjacent right adrenal pheochromocytomas and corrected the elevated urine cortisol (30 micro g/d), epinephrine (0 micro g/d), and calcium (281 mg/d) but not plasma ACTH (125 pg/ml). Neck dissection reduced calcitonin by 96% (5300 to 120 pg/ml) and ACTH by 91% (125 to 11 pg/ml). Carcinoembryonic antigen was reduced from 32.0 to 2.3 ng/ml. Immunohistochemical stain was negative for ACTH in the MTC-positive lymph nodes and the pheochromocytoma. Proopiomelanocortin mRNA by in situ hybridization was positive in the MTC but not in the pheochromocytoma. A repeat pituitary MRI scan was normal. The differential diagnosis of ACTH-dependent Cushing's syndrome in this case included pituitary disease or ectopic ACTH, either from medullary thyroid carcinoma or pheochromocytoma. ACTH stains were unrevealing, but proopiomelanocortin mRNA in situ hybridization in MTC tissue and plasma ACTH response to neck dissection confirmed MTC as the source of ectopic ACTH.

Results of near-total laryngectomy.

The clinical records of 225 patients undergoing primary or salvage near-total laryngectomy (NTL) for laryngeal and pyriform cancer were analyzed for local control and morbidity. If the primary cancer was laryngeal in origin, patients underwent a simple NTL; if it was pyriform, a minor modification called near-total laryngopharyngectomy (NTLP) was used. When NTLP was extended to include necessary portions of the tongue base or posterior pharyngeal wall, pharyngeal reconstructions were added. The principal outcomes studied were 1) 5-year local control of the primary cancer, 2) achievement of lung-powered shunt speech, and 3) incidence of aspiration. The local control of cancer was similar to that expected with total laryngectomy or laryngopharyngectomy. Conversational voice was achieved in 85% of patients surviving beyond 1 year. Some patients required additional surgery for voice -- usually endoscopic dilation. Aspiration was absent if primary healing was achieved. It was troublesome in wound breakdown if the shunt was directly affected. Secondary anti-aspiration procedures were required in 9% of our patients -- usually preserving shunt speech.

Polyethylene oxide gel. A new intranasal dressing after septorhinoplasty.

The purpose of intranasal dressings after septorhinoplasty is to absorb secretions, stop bleeding, act as an internal splint, discourage adhesions, and facilitate nasal hygiene. The ideal dressing should conform to the irregularities of the nasal cavity and resist adherence to the wounds of the nasal lining so it can be easily removed. It should be nonirritating, antiseptic, and economical. The failure of previously reported dressings in one or more of these requirements led us to evaluate a new alternative. Polyethylene oxide gel (Vigilon) was identified as a potential improvement and investigated in 48 patients over 17 months. There were no significant complications. Polyethylene oxide gel appears superior to previously described dressings, primarily because of comfort for the patient at removal.

Utility of near-total laryngectomy for supraglottic, pharyngeal, base-of-tongue, and other cancers.

This report emphasizes the utility of the near-total laryngectomy for those patients in whom conventional conservation surgery is an option but may be oncologically or physiologically unsafe. The near-total operation can be offered for supraglottic cancer, pharyngeal cancer, and, more commonly, primary glottic cancer with cord fixation. Its application is described for patients who are candidates for conventional conservation surgery but are compromised physiologically by age or poor general health and for patients in whom tumor extent would not permit safe conventional conservation surgery.

An endostomal tracheostomy tube for leakproof retention of the Blom-Singer stomal valve.

Patients in whom fistula speech has developed after laryngectomy usually have to occlude their stoma with a finger to talk. This prevents the air that is needed to activate the fistula from being exhaled through the stoma. In order to free the hand, Blom and Singer devised a diaphragm-valved stomal device known as the tracheostoma valve. This device snaps into a flexible retention housing that must be carefully cemented to the skin. Some patients who have undergone near-total laryngectomy (and others with tracheo-esophageal voice prostheses) are unable to use their devices because the moisture and pressure around their stomas break the seal. Since air that is used to produce voice with the valve tends to lift the housing away from the stoma, a better housing design would assure that this motion would improve the seal.

Dedifferentiated acinic cell (acinous) carcinoma of the parotid gland.

Otolaryngologists-head and neck surgeons and surgical pathologists should be aware of the potential for acinic cell carcinomas to dedifferentiate so that adequate histologic tumor sampling can be performed. Dedifferentiated acinic cell carcinoma is a highly malignant, newly recognized parotid malignancy. Clinical clues that may help identify this more ominous lesion must be recognized.

Positive Delphian lymph node: clinical significance in laryngeal cancer.

The significance of metastatic cancer in the Delphian lymph node in patients with laryngeal carcinoma has not been thoroughly discussed. Between 1960 and 1985, we identified 20 cases of histologically proven metastasis to the Delphian node. In 12 of these patients with glottic cancer (T1-T3), the neck was clinically negative but a positive Delphian node was discovered at partial or total laryngectomy; in 6 patients, ipsilateral neck metastasis developed. Eleven of the 20 patients have died from their laryngeal cancer. The frequency of neck metastasis or death, or both, from cancer is unusually high in patients with a positive Delphian node.

Endodermal sinus tumor (yolk sac tumor) of the ear.

Endodermal sinus tumors (yolk sac tumors) are malignant germ cell tumors that usually arise in the gonads. We report what is, to our knowledge, the first known case of an endodermal sinus tumor of the ear. The tumor was present in a developmentally delayed child with an abnormal temporal bone and exhibited histopathologic and immunocytochemical features identical to those of endodermal sinus tumors of gonadal origin. The tumor resolved after chemotherapy, and the patient remained alive without evidence of disease at the time of this writing. The purpose of this report is to add a rare tumor to the differential diagnosis of neoplasms of the ear in children and to familiarize otorhinolaryngologists and head and neck surgeons with its pathologic features and clinical management.

Carotid body tumors: a subject review and suggested surgical approach.

Carotid body tumors are a rare but potentially difficult surgical entity. Their pathology, physiology, and natural history are reviewed along with surgical results reported in the literature. A surgical approach for removal of these tumors is presented which differs significantly from the recommended techniques in that emphasis is placed on intraoperative monitoring of cerebral blood flow, the the selective use of shunts, a tumor-adventitial plane of dissection, preservation of the carotid artery complex, and mobilization of the parotid gland. Thirteen cases using these techniques are reviewed. The mortality rate and the incidence of cerebrovascular sequelae were both 0%. The major morbidity consisted of injury to the lower cranial nerves in five patients (39%) with tumors larger than 5 cm in length.

Surgical management of aneurysms of the distal extracranial internal carotid artery.

Results, complications, and operative techniques of the surgical management of 20 aneurysms of the distal extracranial internal carotid artery (ICA) in 19 patients are reviewed. The proximity of these aneurysms to the styloid process is not considered as a chance occurrence, and the possibility is raised that these lesions are related to trauma from that structure. False aneurysms from spontaneous dissections are believed to occur only in those dissections that begin distally; they are not found in dissections that begin proximally. Treatment was individualized and dependent upon: 1) the size and location of the aneurysm; 2) symptomatology; and 3) hemodynamic considerations based upon intraoperative cerebral blood flow (CBF) measurements determined from the clearance of xenon-133 injected into the ipsilateral ICA. Methods of treatment included: resection of the the aneurysm with placement of an interposition saphenous vein graft in seven patients; resection of the aneurysm with end-to-end anastomosis of the ICA in five; ICA ligation in three; clipping of the aneurysm in one; and extracranial-to-intracranial bypass in four. One patient sustained a postoperative cerebral ischemic complication from embolization which resulted in a mild permanent impairment in right hand dexterity. There were no other cerebral ischemic complications in the group, largely attributable, it is thought, to the use of intraoperative CBF measurements and continuous electroencephalograms. Four patients had transient dysphagia from traction damage to the pharyngeal and superior laryngeal nerves, and one patient with preoperative difficulty in swallowing required a gastrostomy. Long-term results have been excellent. Use of the operating microscope facilitated the suturing of the distal anastomosis in cases in which the ICA was reconstructed by an interposition vein graft or end-to-end anastomosis.

Spontaneous cerebrospinal fluid rhinorrhea: evolving concepts in diagnosis and surgical management based on the Mayo Clinic experience from 1970 through 1981.

Spontaneous cerebrospinal fluid (CSF) rhinorrhea, occurring in the absence of recent trauma, is a distinct clinical entity that can be difficult to diagnose and treat. The case records of 28 patients with this entity were reviewed retrospectively. Pertinent historical data and the results of radiographic evaluation are discussed. The patients were classified into one of three major etiological categories: congenital anomaly, delayed posttraumatic, and tumor groups. The fistulas causing the rhinorrhea were approached extracranially or intracranially, depending on the suspected cause and location. A cure was obtained in 22 of the 27 patients surgically treated (81%). Seventeen patients were cured by a single operation, 5 were cured after multiple operations, and 5 continued to leak despite surgical treatment. Three of the 5 patients who were surgical failures had intracranial tumors, and 2 of them died from complications of persistent fistulas. With the use of a multidisciplinary team approach, an extracranial operation (transethmoidal or transsphenoidal) to repair a spontaneous CSF leak offers a high success rate and is associated with minimal morbidity in appropriately selected patients.

Suitability of near-total laryngopharyngectomy in piriform carcinoma.

The surgical specimens from total laryngectomy and partial pharyngectomy in 20 consecutive cases of piriform sinus carcinoma were studied by whole-organ serial sectioning in the horizontal plane. There had been gross clinical involvement or fixation of the vocal cord in 19 of these cases. Histopathologic evidence of cartilage invasion or piriform apex involvement was found in 17 specimens. In 13 cases, it appeared, retrospectively, that the cancer could have been adequately encompassed without resecting the entire contralateral side of the larynx. When this element is spared, a sphincteric speaking shunt can be made. Careful preoperative assessment would have correctly predicted the suitability or danger of this approach in 18 of the 20 cases; the two indeterminate cases would have required intraoperative quick section. These findings have meaningful implications regarding the scope of conservation surgery in piriform carcinoma.

Deep facial osteology as revealed by coronal sections of the dried skull.

An understanding of the bony framework and relationships of the face is essential to the facial reconstructor. A dried human skull was immersed in a hardening agent to preserve the ethmoidal labyrinth and then cut in the coronal plane to produce six sections. The anterior and posterior surface of each section was photographed and landmarks were labeled. Since the thick anterior bony structures were removed with the first slice, the normal delicate laminae of the deep face, within the deeper sections, were exposed for study.

Lateral rhinotomy. Indications, technique, and review of 226 patients.

Lateral rhinotomy provides excellent exposure of the interior of the nose, the paranasal sinuses, and the nasopharynx with minimal postoperative deformity. A review of 226 lateral rhinotomies performed at the Mayo Clinic, Rochester, Minn, from 1970 through 1979, was made to determine the versatility of this incision and some of its limitations and risks. The suitability of this technique to approach the wide variety of pathologic conditions that occur in the midfacial structures was reaffirmed.

Ectopic internal carotid artery seen initially as middle ear tumor.

An ectopic carotid artery is rare. Its first symptoms may be neurological and could bring the patient to the neurologist during the initial evaluation. However, more often, the neurologist examines a patient in whom serious neurological deficit has occurred after transtympanic exploration of an undiagnosed vascular mass. Because diagnosis can be definitively established by angiography and the consequences of injury to an aberrant carotid artery are serious, operative exploration of vascular middle ear masses probably should be deferred until an ectopic carotid artery has been excluded by angiography.