Onset and Evolution of Clinically Apparent Myasthenia Gravis After Resection of Non-myasthenic Thymomas.

Patients with thymoma and without clinical or electromyographical myasthenic signs may occasionally develop myasthenia several years after thymectomy. Hereby, we investigated the predictors and the evolution of this peculiar disease. We performed a retrospective analysis in 104 consecutive patients who underwent thymectomy between 1987 and 2013 for thymoma without clinical or electromyographic signs of myasthenia gravis. Predictors of post-thymectomy onset of myasthenia gravis were investigated with univariate time-to-disease analysis. Evolution of myasthenia was analyzed with time-to-regression analysis. Eight patients developed late myasthenia gravis after a median period of 33 months from thymectomy. No significant correlation was found for age, gender, Masaoka's stage, and World Health Organization histology. Only high preoperative serum acetylcholine-receptor antibodies titer (>0.3 nmol/L) was significantly associated with post-thymectomy myasthenia gravis at univariate time-to-disease (P = 0.003) analysis. Positron emission tomography was always performed in high-titer patients, and increased metabolic activity was detected in 4 of these patients. Surgical treatment through redo-sternotomy or video-assisted thoracoscopy was performed in these last cases with a remission in all patients after 12, 24, 32 and 48 months, respectively. No patient under medical treatment has yet developed a complete remission. In our study the presence of preoperative high-level serum acetylcholine receptor antibodies was the only factor significantly associated with the development of post-thymectomy myasthenia gravis. The persistence of residual islet of ectopic thymic tissue was one of the causes of the onset of myasthenia and its surgical removal was successful.

Intrasellar Symptomatic Salivary Gland Rest with Inflammations.

BACKGROUND: Instances of ectopic salivary gland tissue within the pituitary gland are rare, they are mostly asymptomatic, and the underlying pathophysiology of symptomatic cases is unclear. We report a case of intrasellar salivary gland rest that presented clinical symptoms and clearly related to inflammatory changes. CASE DESCRIPTION: In the present case, headache, bitemporal hemianopia, and hormone abnormality led to the detection of ectopic salivary gland tissue within the pituitary gland of a 24-year-old man. Imaging revealed a well-circumscribed intrasellar cystic lesion having a diameter of major axis of 16 mm, for which tumorectomy was performed using the nasal approach. The tumor was cystic with stringy content. Pathologic findings revealed that the lesion was composed principally of secretions lacking cell components, whereas the salivary gland tissue was found in the cyst wall. Dilated ducts due to the leakage of secretions were also observed. Acute and chronic inflammation was present around the salivary gland. CONCLUSIONS: Not only are instances of symptomatic ectopic salivary glands rare, but this was also the first case detected to be caused by the pathophysiology involving the leakage of secretions from an ectopic salivary gland and associated inflammation. We report this case to help elucidate the pathophysiology of the condition.

Excision of ectopic mediastinal parathyroid adenoma via parasternal videomediastinoscopy.

Mediastinum is one of the place in which ectopic parathyroid adenomas can be located.Here, an ectopic mediastinal parathyroid adenoma, which was excised via parasternal videomediastinoscopy was presented. The patient with chronic renal insufficiency had increased calcium levels persistence after the surgery for cervical parathyroid adenoma.Radiologic and scintigraphic examinations revealed a focal intense nodule in anterior mediastinum. Parasternal videomediastinoscopy was performed via parasternal incision through the second intercostal space. Ex-vivo specimen radioactivity measurements and frozen examination confirmed parathyroid adenoma. Calcium levels were decreased dramatically after the operation. Parasternal videomediastinoscopy could be an alternative surgical way in anterior mediastinal small masses such as ectopic parathyroid adenoma. It is the first case in which parasternal videomediastinoscopy was used for excision of mediastinal parathyroid adenoma.

Primary hyperparathyroidism: fifth parathyroid intrathymic adenoma in a young patient.

The clinical case described in this paper deals with a young female patient affected by primary hyperparathyroidism caused by an ectopic parathyroid adenoma of a supernumerary intrathymic parathyroid. The patient had hypercalcemia, in association with increased levels of parathormone, but was otherwise asymptomatic. Genetics tests for mutation of the MEN1, HRPT2, and CaSR genes were negative. She therefore underwent laboratory and instrumental tests but localization results in the neck were negative--only an intrathymic nodule was visualized. The complete surgical ablation of the thymus was conducted, which highlighted a nodule that, at histological examination, was shown to be an adenoma of a fifth parathyroid gland. The existence of a fifth, hyperfunctioning, intrathoracic parathyroid appears to be a rare cause of primary juvenile sporadic hyperparathyroidism. This peculiar clinical case could be of interest in similar cases evaluated by other surgeons.

[Mediastinal parathyroidectomy]. / La paratiroidectomia mediastinica.

In most patients the postoperative persistent or recurrent hyperparathyroidism is sustained by parathyroid tissue localized in the mediastinum. A careful preoperative imaging and intraoperative hormone assays are key to reducing the possibility of disease recurrence.

Epidermoid cyst in an intrapancreatic accessory spleen: three case reports and review of the literatures.

The development of an epidermoid cyst in an intrapancreatic accessory spleen is an extremely rare lesion, with only 17 cases being reported in the English literature. All such cases were located in the pancreatic tail, some of which showed carbohydrate antigen 19-9 (CA19-9) immunoreactivity in the lining of the epithelium. A few of them indicated an elevation of the serum CA19-9 level. Here we report three cases of an epidermoid cyst in an intrapancreatic accessory spleen. Cases 1 and 2 were 57-year-old and 70-year-old women, while case 3 was a 37-year-old man. All three cases were asymptomatic. Serum CA19-9 levels showed within normal limits (case 1), slightly elevated (case 2), and clearly elevated (case 3). They underwent a distal pancreatectomy with splenectomy (cases 1 and 2) and without splenectomy (case 3). Grossly, the surgical specimen was a well-demarcated, multiple (case 1) or solitary (cases 2 and 3) cystic mass in the pancreatic tail. A high level of fluid CA 19-9 was detected in case 1. Microscopically, the cystic walls were lined with squamous and cuboidal epithelium, which were surrounded by normal splenic tissue and hyalinized fibrous tissue. The lining squamous epithelium was revealed as nonkeratinizing (Cases 1 and 2) or keratinizing (Case 3). Immunohistochemically, CA19-9 was positive in the monolayer and surface layer of the cuboidal epithelium, but negative for the keratinizing squamous epithelium. As for the histogenesis, it is suggested that the cystic lining of the epithelium may derive from the pancreatic duct which protrudes into the accessory spleen.

[Role of minimal invasive surgery for primary and secondary hyperparathyroidism]. / Stellenwert der minimal-invasiven Chirurgie beim primären und sekundären Hyperparathyroidismus.

BACKGROUND: The standard surgical approach to treat primary (pHPT) and secondary hyperparathyroidism (sHPT) used to be a cervicotomy with exploration of all four parathyroid glands. This access has been challenged recently by the introduction of minimally invasive techniques in order to achieve superior cosmesic results and to reduce theatre time. We analyzed the advantages and morbidities of these surgical aproaches. PATIENTS AND METHODS: Between 1997 and 2006 a total of 123 patients (109 with pHPT and 14 with sHPT) underwent parathyroidectomy at the ENT Department in Luzern. Ultrasonographic scanning was performed on 74 patients (68%), szintigraphy in 8 patients (7%) and both scanning methods in 27 patients (25%). 103 patients were available for follow-up. The indications for each technique were reviewed and outcome measures included serum Calcium and parathyroid hormone levels. RESULTS: Sensitivity for preoperative ultrasonographic and scintigraphic scanning was 67% and 65% for identification of the correct quadrant and 74% and 71% for identification of the correct side. A bilateral exploration was performed until June 2001 for all patients. Thereafter, a minimally invasive approach was chosen for patients with pHPT, whereas patients with sHPT still require bilateral exploration. Adequate preoperative localization was a prerequisite for a minimally invasive technique. Mean postoperative serum Kalzium levels were within the normal range, independently of the surgical technique and disease. Two patients developed hypercalcemia after an initially successful operation. CONCLUSIONS: Review of the literature confirms the shift from bilateral exploration towards minimally invasive techniques. The incidence of persistent or recurrent disease as well as the rate of complications seems comparable. Operation time for minimally invasive techniques is reduced in the hands of an experienced surgeon. However, proper preoperative localization of the diseased parathyroid gland is not always possible and the expenses of intraoperative parathyroid hormone measures do not lower the overall costs. Considerable experience and a multidisiplinary approach (endocrinologist, surgeon, pathologist) is required to adopt efficient minimally invasive techniques. As for sHPT, bilateral exploration remains the treatment of choice.

Outpatient video-assisted thoracoscopic surgery (VATS) for ectopic mediastinal parathyroid adenoma: a case report and review of the literature.

Up to 20% of abnormal parathyroid glands causing primary or secondary hyperparathyroidism are located ectopically. Of these, approximately 1%-2% reside in the mediastinum and may not always be resectable through a traditional cervical approach. Recently, video-assisted thoracoscopic surgery (VATS) has arisen as a minimally invasive method for resecting mediastinal parathyroid glands and avoiding the complications and higher morbidity of a sternotomy. In this paper, we present a case of a patient with asymptomatic hyperparathyroidism and an ectopic parathyroid on sestamibi imaging in the mediastinum. Initially, the patient underwent a bilateral cervical exploration, left upper parathyroidectomy, and partial thymectomy; however, postoperatively, he continued to have persistently elevated serum calcium and parathyroid hormone levels. Ultimately, management consisted of parathyroidectomy through a VATS approach, along with intraoperative parathyroid assay monitoring and frozen-section pathologic analysis. The patient was successfully discharged to home several hours after surgery. To our knowledge, this is the first reported case of mediastinal ectopic parathyroid adenoma treated with outpatient VATS.

Hormonal, pituitary magnetic resonance, LHX4 and HESX1 evaluation in patients with hypopituitarism and ectopic posterior pituitary lobe.

OBJECTIVE: LHX4 and HESX1 are important in early stages of pituitary development and their mutations can be associated with an ectopic posterior lobe (EPL) in the pituitary of patients with hypopituitarism. The EPL can be located at the median eminence or at the path of the pituitary stalk. The aim of this study was to analyse LHX4 and HESX1 and characterize the hormonal deficiency profiles, establishing relationships with magnetic resonance imaging (MRI) findings in these patients. PATIENTS AND DESIGN: Sixty-two patients with hypopituitarism associated with EPL were submitted to evaluation of pituitary function, analysis of MRI with EPL location and molecular analysis of LHX4 and HESX1 using polymerase chain reaction (PCR), digestion with restriction enzyme and automatic sequencing. RESULTS: Forty-two patients had a nonvisualized pituitary stalk (NPS), and 20 a visualized pituitary stalk (VPS). Most patients (95%) with NPS had combined pituitary hormone deficiency (CPHD), with ACTH deficiency in 85%. In patients with VPS, CPHD was found in 50% and ACTH deficiency occurred in only 20%. The frequency of the location of EPL was similar in patients with VPS and NPS: 35% at median eminence and 65% at the path of the stalk. No mutations in LHX4 and HESX1 were identified. Three new polymorphisms in LHX4 were found. CONCLUSIONS: ACTH deficiency is frequent in patients with hypopituitarism and NPS (85%), the location of EPL at the median eminence was not predictive of the hormonal profile [isolated GH deficiency (IGHD) or CPHD], and LHX4 and HESX1 genes mutations remain rare causes of hypopituitarism associated with EPL.

Torsion of an ectopic liver in a young child.

A large ectopic liver nodule connected to the greater omentum had undergone infarction and separation because of torsion of its connecting stalk and was the cause of acute abdominal pain and elevated liver enzymes in a young female child. At laparotomy for a suspected perforated appendicitis, a hemorrhagic peritoneal fluid was found, together with an intraperitoneal solid mass lying freely in the right iliac fossa. The definitive diagnosis was obtained by histopathology. This is the first known such reported case.

Supernumerary ovary found by ultrasonogram and FSH measurement after an extensive operation for a yolk sac tumor of the ovary.

A rare case of supernumerary ovary found by a transvaginal ultrasonogram and follicle-stimulating hormone (FSH) measurement is presented. The patient was a 32-year-old female who underwent an extensive operation for a yolk sac tumor of the ovary. An asymptomatic cystic tumor was found during follow-up. There was no evidence of recurrence or metastasis of the yolk sac tumor. Although histological confirmation was not possible because the patient refused removal of the mass, a diagnosis of supernumerary ovary was made because changes in the shape of the cystic mass completely correlated with changes in the serum FSH level.

Repeated 131I treatment of a residual ovarian teratoma containing malignant thyroid tissue.

A 49-year-old woman with ovarian teratoma received 131I treatment three times for an unresectable mass containing malignant thyroid tissue after surgery. Repeated 131I treatment effectively reduced serum thyroglobulin (Tg) level and tumour uptake of 131I, despite absence of any change in size of the treated tumour. Treatment did not inhibit the increase of serum CA-125 and tumour 201Tl uptake, associated with progression of a radioresistant intratumoral hyper-perfused tissue component, detected by colour Doppler ultrasound. Serum CA-125 level and tumour 201Tl uptake were not significantly changed despite temporary increases in serum Tg level after each 131I treatment. These observations indicate the importance of diagnostic measures using combined functional imaging and tumour markers in managing this rare tumour.

Lingual thyroid presenting as acquired hypothyroidism in the adulthood.

There are numerous reported cases of lingual thyroid with an obvious prevalence in pediatric age. Such ectopic thyroid glands are probably quantitatively deficient and thyroid function may be low or at a low normal level. Apparently, most cases of ectopic thyroid tissue develop congenital hypothyroidism, the so-called cretinism. In this report, we describe a very rare adult male case of lingual thyroid who developed hypothyroidism in adulthood; the anomaly remained undiscovered, being without local common symptoms, and permitted a normal life.

Frequency and characteristics of lingual thyroid not detected by screening.

Four patients with lingual thyroid glands presenting beyond the neonatal period have been evaluated at the Hospital for Sick Children, Toronto since the advent of neonatal TSH screening. All were female, clinically euthyroid at diagnosis and presented with symptoms of a lingual mass. We estimate that 1.6% of lingual thyroids are missed by this TSH based thyroid screening program and approximately 1/600,000 live births present in childhood or adolescence with a lingual thyroid. Physicians should still include lingual thyroid in the differential diagnosis of a mass at the base of the tongue.