Spontaneous rupture of a parathyroid adenoma presenting as a massive cervical hemorrhage: report of a case.

We report an unusual case of spontaneous rupture of a parathyroid adenoma causing cervical hemorrhage. A 60-year-old woman presented to our hospital after the sudden development of extensive ecchymosis of her neck and upper anterior chest wall. Computed tomography (CT) scanning revealed a hematoma in the left retrotracheal space, and laboratory examinations revealed significant hypercalcemia, hypophosphatemia, and a high level of intact parathyroid hormone. Primary hyperparathyroidism was diagnosed, but it was not until the hematoma had subsided, 4 months after her initial presentation, that a parathyroid adenoma was revealed by CT. An operation was performed, and a parathyroid adenoma with hemosiderin deposition was histologically diagnosed. Although this phenomenon is unusual, all endocrine surgeons should be well aware of the possibility of its occurrence.

Recovery of parathyroid function after total thyroidectomy.

To prevent postoperative hypoparathyroidism following total thyroidectomy, the parathyroid glands are preserved in situ and/or resected or devascularized parathyroid glands are autotransplanted. We conducted a retrospective investigation utilizing biochemical and specific endocrine assessments to evaluate the difference in recovery of parathyroid function between the two operative methods. A total of 92 patients underwent total thyroidectomy at our hospital during the period between 1990 and 1997. These patients were divided into a preservation group (n = 83), with one or more preserved glands in situ, and an autotransplantation group (n = 9), with only transplanted glands. The level of intact parathyroid hormone (PTH) was completely restored by 1 year postoperatively in 83% (69/83) of the preservation group patients. In the remaining 14 patients (17%), the intact PTH had fallen below detectable levels on postoperative day (POD) 1, then subsequently recovered to 70% of the preoperative levels. Comparatively, in the autotransplantation group, the mean level of intact PTH recovered to only 43% of the preoperative levels. The results of this study suggest that parathyroid glands should be preserved in situ whenever possible, and that when intact PTH levels fall below detectable limits on POD 1, they may never recover to the preoperative levels in those patients.

Successful autotransplantation of an adrenal gland using a new method of omental wrapping: report of a case.

Bilateral total adrenalectomy results in the need for patients to take lifelong supplements of adrenal steroids, with the risk of possible Addisonian crisis. Few reports of the successful autotransplantation of adrenal tissue in muscular pockets have been documented; however, we describe herein the case of a 22-year-old woman in whom autotransplantation of an adrenal gland was successfully performed employing a new method of omental wrapping. The patient underwent bilateral total adrenalectomy for bilateral pheochromocytoma at which time adrenal tissue was sliced into 1-2-mm thick pieces, half of which were placed in muscular pockets in the abdominal rectus muscles, and the remaining half put onto the omentum and wrapped with it. Laboratory examinations done 6 months after surgery showed recovery of her adrenal function, and 4 months later steroid supplements were able to be discontinued. Scintigraphic studies using [131I]-iodomethyl-norcholesterol ([131]I-Adosterol) demonstrated clear activity in the omentum, weak activity in the rectus muscles, and no activity in the adrenal beds. These findings suggest that the omentum may be more suitable as an implantation site for adrenal tissue than muscular pockets.

[A case of stage IV breast cancer showing long-term complete response to combination therapy with 5'-DFUR and MPA].

We report a 62-year-old woman with supraclavicular lymph node, pleural and bone metastases from breast cancer showing a long-term complete response to combination therapy with 5'-DFUR and MPA. A large amount of pleural effusion was drained followed by administration of ADM, which improved the amount of effusion. Treatment with CAF and TAM decreased tumor size, but CAF was abandoned due to severe leukopenia. Mastectomy was performed for local control. However, levels of tumor markers increased progressively. Administration of CMF was tried, but tumor markers continued to increase. Therefore, combined chemoendocrine therapy with 5'-DFUR and MPA was undertaken. Levels of tumor markers normalized and a complete response was obtained 13 months after starting this combination therapy. There are no further metastatic lesions evident, and this status has been consistently maintained for more than three years (six years and five months after diagnosis of breast cancer). There were no significant side effects of this combination therapy except for mild weight gain and moon face. This combination regimen with 5'-DFUR and MPA is considered useful as a second-line treatment for advanced breast cancer.

Iodine-131 MIBG imaging in multiple endocrine neoplasia type 2B.

A 16-year-old boy had a swollen neck that was a result of multiple endocrine neoplasia Type 2B (MEN 2B). CT revealed bilateral thyroid tumors, swelling of right cervical lymph nodes, and slight enlargement of the right adrenal gland. I-131 metaiodobenzylguanidine (MIBG) scintigraphy demonstrated increased uptake in the right adrenal gland and the left thyroid tumor, but no abnormal uptake in the right thyroid tumor and the right cervical lymph nodes. Postoperative pathologic findings were consistent with the diagnosis of right adrenal medullary hyperplasia, which is a precursor of pheochromocytoma. In patients with MEN 2B, I-131 MIBG scintigraphy in conjunction with CT of the adrenal glands should be performed to determine the disease stage of the adrenal medullae. In the cervical region, the diagnosis was medullary thyroid carcinoma (MTC) in both thyroid tumors and metastases in the right cervical lymph nodes. The right MTC was more aggressive than the left MTC. It is interesting that not all sites of known MTC take up I-131 MIBG to the same degree.

The role of ansa-to-recurrent-laryngeal nerve anastomosis in operations for thyroid cancer.

OBJECTIVE: To study the recovery in phonation after reconstruction of the recurrent laryngeal nerve (RLN) in patients whose thyroid cancer was invading the nerve, and to evaluate the role of ansa cervicalis to RLN anastomosis (ARA) in operations for thyroid cancer. DESIGN: Retrospective study. SETTING: University hospital and private thyroid clinic hospital, Japan. SUBJECTS: 34 patients with thyroid cancer who underwent reconstruction of unilateral RLN and 331 consecutive patients operated on for thyroid cancer. INTERVENTIONS: Reconstruction was direct anastomosis (DA), free nerve grafting (FNG), vagus-RLN anastomosis (VRA) or ARA, including anastomosis behind the thyroid cartilage. MAIN OUTCOME MEASURES: Maximum phonation time (34 normal subjects and 26 patients with vocal cord paralysis served as controls), laryngoscopic examination, and the ratio of reconstruction in patients who needed resection of the RLN. RESULTS: The maximum phonation time started to increase rapidly 2-5 months postoperatively in most cases as the patients' voices recovered, and 12 months after reconstruction was significantly longer than in those patients with vocal cord paralysis (P < 0.0001). It was comparable to that of the normal subjects, although the reinnervated cords were fixed in the median. The number of reconstructions in the series of 331 patients increased from 18% to 82% after we started doing ARA with the meticulous technique of anastomosis inside the thyroid cartilage. CONCLUSIONS: ARA is as effective as DA or FNG in improving phonation in patients who need resection of a unilateral RLN. As ARA has several advantages over FNG it has a definite place in operations for thyroid cancer.

A family of MEN1 with a novel germline missense mutation and benign polymorphisms.

The gene responsible for multiple endocrine neoplasia type 1 (MEN1) has recently been cloned, and its germline mutations were identified in patients with this syndrome. The majority of the mutations, frameshift or nonsense mutations, are expected to result in a loss of function of the gene product menin. Since the consequence of less common missense or in-frame deletion mutations is not clear, careful judgment is necessary regarding the role(s) of such mutations in MEN1 disease. Here we describe a large multigenerational MEN1 family with a novel germline missense mutation and three benign polymorphisms. The proband was a man with hyperparathyroidism and thymic carcinoid. We performed biochemical studies and DNA analyses of the MEN1 gene simultaneously and independently as family screening studies. Seven patients including the proband were identified, and all of them carried a heterozygous germline missense mutation E45G, but 5 members with normal biochemical results did not. This mutation was not observed in 50 normal volunteers. This novel missense mutation is therefore almost conclusively responsible for the disease. Although all of the mutant gene carriers in the present study already had clinical diseases, an MEN1 gene analysis in younger individuals at risk would be very useful in identifying carriers before the onset of the symptoms.

Ansa-recurrent nerve anastomosis for vocal cord paralysis due to mediastinal lesions.

Recurrent laryngeal or vagus nerve injuries in the mediastinum are repaired rarely because of technical difficulties. Impairment in phonation is especially severe in patients with respiratory dysfunction. We performed a simple and less invasive reconstruction, ansa cervicalis-recurrent laryngeal nerve anastomosis in the neck, to improve phonation in 2 patients. Although the reinnervated vocal cord did not regain normal movement, both of the patients obtained excellent improvement in phonation.

[Treatment of recurrent laryngeal nerve paralysis by several types of nerve suture].

Although simple neurorrhaphy of the injured recurrent laryngeal nerve usually results in impaired movements of the vocal cord because of misdirected regeneration, phonation recovers because the vocal cord maintains tension during phonation. Simple neurorrhaphy is possible in only a few of patients who have had their nerve severed because of thyroid surgery for thyroid cancer. We tried free nerve grafting, vagus nerve-recurrent nerve suture and ansa cervicalis-recurrent nerve suture as well as the simple neurorrhaphy in 3, 1, 1 and 3 patients, respectively. All of the 8 patients who had repair of the recurrent nerve recovered from hoarseness. Maximum phonation times of these patients ranged from 17 to 41 sec with a mean of 26.5 +/- 9.6 sec, which were significantly longer than those of 23 patients without nerve repair. Each modality of the repair obtained similar good results, although the patient who underwent vagus-recurrent suture required a longer period for recovery. These results indicate that not only simple neurorrhaphy of the recurrent nerve but also free nerve grafting, vagus nerve-recurrent nerve suture, or ansa cervicalis-recurrent suture are effective in recovery of the phonation in patients with peripheral and unilateral recurrent nerve paralysis.