Familial papillary carcinoma of the thyroid: a report of nine first-degree relatives of four families.

The authors report the occurrence of papillary carcinoma of the thyroid in nine first-degree relatives of four families among a consecutive series of 97 patients with papillary carcinoma of the thyroid who were operated on from 1991 to 1998. Total thyroidectomy was performed in all cases. All patients are alive without evidence of disease after a mean follow-up period of 43 months. Since in our series familial papillary carcinoma of the thyroid was found in 9.3% of patients, we suggest an adequate screening among first-degree relatives of all patients with papillary thyroid carcinoma. Because of reported aggressive behaviour of familial papillary carcinoma of the thyroid, aggressive surgical treatment plus post-operative thyroid remnant ablation with radio-iodine should be warranted in all patients.

[The inferior nonrecurrent laryngeal nerve: a report of 7 cases observed since 1987]. / Il nervo laringeo inferiore non ricorrente: descrizione di 7 casi osservati dal 1987.

The non-recurrent inferior laryngeal nerve is a rare anomaly (0.34% on th right side, extremely rare on the left side) that increases the risk of damage to the nerve during thyroid and parathyroid surgery. Seven cases of non-recurrent laryngeal nerve are reported, emphasizing the diagnostic accuracy of angio-MR for the anatomical identification of the vascular anomaly that invariably occurs with the nerve malformation.

Surgical treatment of paragangliomas of the carotid body and other rare localisations.

BACKGROUND: Cervical paragangliomas are uncommon benign or malignant neoplasms, deriving from stem cells of the neural crest. Compared to all the tumors of the head and neck, they occur less frequently. They can be found in any part of the body where there are sympathetic ganglia including chemoreceptors, suprarenal medulla, retroperitoneal ganglia and the extreme branches of the vagus nerves. It is not easy nowadays to define properly their biological behaviour, the possible multicentric location and the association with Multiple Endocrine Neoplasms; this is considered particularly important and occurs in 42 per cent of the cases of familial neoplasms of the paraganglion system. METHODS: After a review of recent diagnostic, pathological and clinical findings, the authors report their experience, between 1970-1995, of 10 patients affected by sporadic paraganglioma and 1 by familial multicentric neoplasm localised in the carotid bodies of both sides, left vagus nerve and left hypoglossus nerve. All patients but one were treated by a curative resection of the neoplasm. In one case only an explorative laparatomy was possible because of visceral and vascular involvement. RESULTS: There is no mortality. There are no modifications in arterial blood pressure and catecholamine values in all patients. The complications were a recurrential palsy in a patient operated on for vagal paraganglioma; a recurrential palsy and temporary dysarthria in the patient affected by multiple familial paraganglioma; another patient operated on for carotid body paraganglioma showed a cerebral ischemic lesion which caused a slightly transitory facial-brachial motor deficit on the right side and speech impairment. CONCLUSIONS: We can venture to say that any type of cervical, mediastinal or retroperitoneal swelling in persons belonging to a genetically prone family must be first of all considered a possible paraganglioma. For this reason the patient with more than one growth of this type, whether synchronous or not, must undergo genetic investigation, along with the rest of his family.

Insular carcinoma of the thyroid. A report of 8 cases.

Insular carcinoma of the thyroid (ICT) is an uncommon malignancy with intermediate morphology and behaviour between well-differentiated and anaplastic thyroid carcinoma. Eight patients with ICT underwent total thyroidectomy. A modified neck dissection was carried out in six of them. Cervical lymph nodes metastases were detected during surgery in six patients or at scintigraphy in two patients who did not undergo neck dissection. Postoperatively, a patient developed diffuse metastases not detected by 131I whole-body scintigraphy and she died of disease 6 months later despite radio- and chemotherapy. Another patient had distant metastases detected by 131I whole-body scintigraphy and successfully treated by radioiodine ablative therapy. Unfortunately, she developed other distant metastases with no 131I uptake and died of disease 23 months later despite chemotherapy. After a mean follow-up of 5.5 years, 6 patients (75%) were alive without evidence of disease. These observations confirmed the aggressiveness of ICTs that sometimes are not responsive to current available therapies. The frequent occurrence of metastases to the regional lymph node calls on for a modified neck dissection in all patients with ICT.

Paragangliomas of the carotid body and other rare localisation.

Cervical paragangliomas are uncommon benign or malignant neoplasms, originated by stem cells of neural crest. It is not easy nowadays to define properly their biological behaviour, the possible multiple location and the association with Multiple Endocrine Neoplasms. After a wide review about recent diagnostic, pathological and clinical acquisition, authors report their caseload of 10 patients affected by sporadic paragangliomas and 1 by familial multiple neoplasm localised in carotid bodies of both sides, left vagus nerve and left hypoglossus nerve. All patients but one were treated by a curative resection of the neoplasm. In one case only an explorative laparatomy was possible because of the visceral and vascular involvement.

Surgical treatment of paragangliomas of the neck.

Of a total of 5,700 surgical procedures on the neck performed at our Institution between 1984 and 1995, 13 operations (0.22%) have been done on 11 patients with 16 cervical paragangliomas. A woman underwent resection of synchronous bilateral carotid body tumours and of an intravagal paraganglioma. Ten years later, after preoperative angiographic embolization, she underwent resection of a paraganglioma of the left hypoglossal nerve. Her sister, at age 21, underwent resection of a carotid body tumour and, respectively 19 and 20 years later, of a right and left intravagal paraganglioma. An interposition graft for replacement of the carotid bifurcation was necessary in one patient only. During resection of a left carotid body tumour, acute hypotension occurred resulting in an ischemic lesion of the right temporal lobe. Postoperatively, she also complained of respiratory distress that responded to medical therapy with difficulty. The related neurologic symptoms completely resolved three months after surgery. The operation for a paraganglioma of the left hypoglossal nerve resulted in a temporary motor deficit of the tongue and in permanent considerable difficulty in swallowing. Unilateral recurrent nerve palsy occurred in two patients. No patients during the postoperative follow-up showed signs of local recurrence or metastatic disease. In conclusion, surgery is an effective treatment for cervical paraganglioma, but because of the high surgical complication rate, an experienced and skilled surgeon is called on to optimize outcome. An adequate perioperative care is advisable.

[Paraganglioma in the case records of the III Surgical Clinical Institute]. / I paragangliomi nella casistica dell'istituto di III Clinica Chirurgica (1970-1995).

Cervical paragangliomas are uncommon benign or malignant neoplasms, originated from the stem cells of the neural crest. Up to date it is still not easy to define properly their biological behaviour, the possible multicentric location and the association with Multiple Endocrine Neoplasias. After a wide review of recent diagnostic, pathological and clinical notions, the Authors report their experience, from 1970 to 1995, of 10 patients affected by sporadic paraganglioma and 1 by familial multicentric neoplasm localized on carotid bodies of both sides, left vagus nerve and left hypoglossus nerve. All patients but one were treated by a curative resection of the neoplasm; in one case only an explorative laparotomy was possible because of the visceral and vascular involvement.

[Total thyroidectomy technique: suggestions and proposals of surgical practice]. / Tecnica della tiroidectomia totale. Suggerimenti e proposte di pratica chirurgica.

Total thyroidectomy at present depicts a diffuse surgical procedure in the management of benign and malignant disease of thyroid gland. It is followed by a low incidence of iatrogenic damages (nervous lesions or permanent hypoparathyroidism), just like subtotal thyroidectomy and lower than surgery for nodular recurrences. Authors present the surgical technique they follow to perform total thyroidectomy, used in over 400 cases of benign thyroid diseases operated since 1986. The most important points of this surgical procedures are represented by exposure and sparing of inferior laryngeal nerve and by preservation of parathyroid function. Parathyroid glands can be exposed to direct surgical trauma but, more often, they are injured by damage of their vascular supply. To avoid this complication, vascular ligations of inferior thyroid artery have to be done never on the trunk of the artery, but on its branches just near the glandular capsula. Sparing of inferior laryngeal nerves comports the exposure of this structure for all its cervical course especially in the terminal edge, when the nerve is nearest to the gland. Systematical application of illustrated procedure has produced no operative mortality, no inferior laryngeal nerve permanent palsy, transient hoarseness in 0.5%, and transient symptomatic hypocalcemia in 2.7%.

[Current diagnostic-therapeutic trends in carcinoids of the papilla of Vater]. / Moderni orientamenti diagnostico-terapeutici sui carcinoidi della papilla di Vater.

Diagnosis and treatment of carcinoid tumor of the ampulla of Vater: case report. The carcinoid tumour of the ampulla of Vater is extremely rare. The authors report one case. Therefore, they dwell upon the problem connected with classifications, symptomatology and diagnosis of these neoplasms which present difficulty to diagnose preoperatively. At last, they reaffirm the primary role of pancreaticoduodenectomy with lymphadenectomy and, if it is possible, the necessity to extirpate the liver metastases.

[The Soupault-Bucaillle operation in the treatment of Billroth II gastric resection syndrome]. / L'intervento di Soupault e Bucaille nel trattamento della sindrome da gastroresezione sec. Billroth II.

The study analyzes the benefits related to the gastroduodenojejunoplasty (GDJP) according to Soupault & Bucaille, adopted as the corrective treatment of the Billroth II gastric resection syndrome in those cases resistant to the dieting-pharmacological supports. Our experience is based on 18 cases (13 males, 5 females, mean age 39 yrs), treated from 1982 to 1987 with GDJP for a gastric resection syndrome. The patients were observed 5 years after their resection: 10 presented with an atrophic gastritis/gastric atrophy and all had a diffuse acute phlogosis in presence of biliary storage. Manual anastomoses were performed in the first 8 cases, while staplers were used in the remaining cases. Results were quite satisfying; no operative mortality or specific complications, nor pain and biliary vomiting were registered; 14 patients over 18 (77%) gained weight significantly; only 1 patient reported low-intense early "dumping-like" symptoms easily controlled by medical therapy. Endoscopy showed no biliary reflux in all cases. Biopsies of the stump, carried on before and after the GDJP intervention, showed a marked reduction of the inflammatory aspects in 76% (13) of the patients. Among the 10 patients with atrophic gastritis/gastric atrophy, only 2 (20%) presented a significant increase in the number of gastric parietal cells and gastric principal cells.

[Anastomosis or closure of the pancreatic stump after duodenopancreatectomy?]. / Anastomosi o chiusura del moncone pancreatico dopo duodenocefalopancreatectomia?

From 1986 to June 1990, 10 pancreaticoduodenectomies were performed in our Department (VIII Patologia Chirurgica, Università "La Sapienza", Roma). The reconstructive sequence was based on the pancreaticojejunal anastomosis (2 end-to-end pancreaticojejunostomies after invagination, 3 end-to-side Wirsung jejunostomies) in 5 patients. The pancreatic stump was sutured using a 50mm linear stapler in the remaining 5 patients. There was a high incidence (75%) of fistulization after direct suture of the pancreatic stump, although related mortality was null. Among patients treated with pancreaticojejunal anastomosis, a fistula on the end-to-end pancreaticojejunostomy with exitus of the patient, was registered in 1 case. On principle, we believe a end-to-side Wirsung jejunostomy should be performed after pancreaticoduodenectomy. When a safe anastomosis of the pancreatic stump cannot be performed (because of the soft parenchyma, easily lacerable) the technique of the direct suture with stapler is advisable.