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[A rare case of ectopic, normally functioning thyroid tissue presenting as a left submandibular mass].

This report describes the case of a 53-year-old female, who presented with a painless swelling in the left submandibular region which had gradually increased in size. Thirty years ago, this patient underwent subtotal thyroidectomy at another hospital and her thyroid function subsequently appeared normal. She presented no symptoms of hypothyroidism when she visited our hospital. A clinical diagnosis of a benign tumor arising from the submandibular gland was made with enhanced CT scan, MRI and technesium scintigraphy. The submandibular mass was surgically excised and the resected tissue revealed a well-delineated, encapsulated, solid and reddish-brown colored mass. Histological examination confirmed ectopic thyroid tissue with partial adenomatous goiter including vacuolated rich colloid in the thyroid follicles which suggested compensatory hyperplasia. Because this patient presented with post-operative hypothyroidism, we concluded that this ectopic lesion had continued to secrete thyroid hormone. Ectopic thyroid tissue should be considered in the differential diagnosis of swellings involving the submandibular area, especially if the ectopic tissue would be the only functioning thyroid tissue.

Choristoma↗

Localization of ectopic corticotropin-producing carcinoid tumor with use of indium-111 pentetreotide scintigraphy.

OBJECTIVE: To report on the diagnosis of ectopic corticotropin (adrenocorticotropic hormone [ACTH])-producing bronchial carcinoid tumor by indium-111 pentetreotide (octreotide scan) scintigraphy. METHODS: We present a case of ectopic ACTH syndrome caused by an occult bronchial carcinoid tumor arising in a lymph node and review the pertinent literature. RESULTS: Biochemical diagnosis of ACTH syndrome can be difficult, and conventional imaging modalities often do not demonstrate these small carcinoid tumors. After biochemical proof of the presence of ectopic ACTH syndrome in our patient, conventional radiographic studies did not demonstrate any lesions. An octreotide scan showed a lesion in the lung, which was confirmed surgically. ACTH values returned to normal after resection of the lesion, and octreotide scans confirmed the completeness of surgical resection. The carcinoid tumor originated in a lymph node outside the bronchus. The differential diagnosis of ACTH syndrome, the localization of ectopic ACTH-producing tumors, the bronchial carcinoids, and the uniqueness of the carcinoid tumor arising in a lymph node are briefly discussed. CONCLUSION: Octreotide scintigraphy is useful in localizing occult carcinoid tumors and can be used in the follow-up of patients after successful removal of these tumors.

Journal Article↗

Cushing's syndrome from ectopic production of corticotropin by a metastatic gastrinoma.

OBJECTIVE: To present a case of a young woman with new-onset diabetes mellitus resistant to insulin attributable to Cushing's syndrome caused by ectopic production of corticotropin by a metastatic gastrinoma. METHODS: We summarize the clinical presentation and the pertinent laboratory values in a patient with Cushing's syndrome. A review of the literature regarding ectopic production of corticotropin by gastrinomas is also presented. RESULTS: A 26-year-old woman with dehydration, severe hyperglycemia, and hypokalemia was seen in consultation. The patient required large doses of insulin to control plasma glucose, and further work-up confirmed the presence of Cushing's syndrome caused by ectopic production of corticotropin from a metastatic gastrinoma. CONCLUSION: This case is unusual in that the patient was relatively young and the clinical presentation of Cushing's syndrome was dominated by uncontrolled diabetes, insulin resistance, and hypokalemia. At the time of this diagnosis, the patient already had evidence of multiple liver metastatic lesions from a pancreatic gastrinoma. The rapid occurrence of difficult-to-treat diabetes and hypokalemia should raise the suspicion of Cushing's syndrome from ectopic production of corticotropin. In fact, patients with metastatic pancreatic tumors and poorly controlled diabetes with hypokalemia should undergo evaluation for Cushing's syndrome, even in the absence of the typical stigmas, because of rapid development of the disease and high levels of corticotropin.

Adenoma, Islet Cell↗

Ectopic localization of tungiasis.

Tungiasis is caused by the penetration of the female sand flea Tunga penetrans into the epidermis. It is generally assumed that lesions are confined to the feet. To determine to what degree tungiasis occurs at other topographic sites, 1,184 inhabitants of a poor neighborhood in northeastern Brazil were examined; 33.6% were found to have tungiasis (95% confidence interval = 30.9-36.4%). Six percent presented lesions at locations other than the feet, with the hands being the most common ectopic site (5.5%). Other sites were the elbows, thighs, and gluteal region. Ectopic tungiasis was significantly associated with the total number of lesions (P < 0.001) and an age less than 15 years old (P = 0.02). In 86 patients actively recruited with lesions on their feet, ectopic localizations were observed in 25.6%. Since untreated sand flea lesions are prone to become superinfected, clinicians should be aware of not missing any ectopic localization of tungiasis.

Adult↗

Ectopic decidua of pelvic lymph nodes: a potential diagnostic pitfall.

Ectopic decidua is one of several benign lymph node inclusions that have been increasingly documented in the literature, most often in postmortem examinations of pregnant woman and recently in pregnant women with cervical squamous cell carcinoma. Although lacking clinical significance of its own, the major diagnostic implication would be misdiagnosis as metastatic carcinoma in the lymph node. Intraoperative frozen sections are often performed prior to radical hysterectomy, leading to a potential alteration of therapy if metastatic carcinoma is identified in the lymph nodes. We report such a case of a pregnant woman with cervical squamous cell carcinoma requiring lymphadenectomy and hysterectomy, in which the intraoperative frozen section of a pelvic lymph node with ectopic decidual change was mistakenly identified as metastatic carcinoma. Its histologic resemblance to carcinoma and location within subcapsular sinuses, compounded with the fact that ectopic lymph node decidua is not commonly seen in routine practice, can lead to this diagnostic pitfall. We review the literature regarding ectopic decidua, its presence in lymph nodes, and its pathogenesis, as well as review the literature on benign lymph node inclusions.

Adult↗

[Ectopic ureter in 54 children].

PURPOSE: We reviewed 54 pediatric patients with ectopic ureter treated at our institution from 1975 to 1999. MATERIALS AND METHODS: Our series comprised 40 female and 14 male children, with age from 1 month to 11 years. Clinical records of the patients were reviewed retrospectively. RESULTS: Chief complaint was urinary incontinence in 24, high fever in 18, abdominal mass in 6, scrotal swelling in 3 and growth retardation in 2 patients. Two patients were found to have ectopic ureters without symptom during urological work-ups for their anorectal anomaly. The ectopic ureters opened into vagina in 19, vestibulum in 8, bladder neck in 7, urethra in 17, seminal vesicle in 2 and ejaculatory duct in 1 patient (s). Treatment was ureterocystoneostomy in 30, nephroureterectomy in 19, hemi-nephroureterectomy in 2, and ureteral ligature in 1 patient (s). Postoperatively, most of the patients became symptom free except for 6 patients in whom urinary incontinence was not cured due to mal-development of the bladder neck and sphincter, and due to Gartner's duct cyst. CONCLUSION: Urinary incontinence and urinary tract infection are most frequent presentations of ectopic ureter in children. Although most of the patients are cured with ureterocystoneostomy or nephroureterectomy, some incontinent girls continue to have urinary incontinence due to mal-development of the bladder neck and sphincter or Gartner's duct cyst.

Child↗

Comparison of flecainide with quinidine for suppression of chronic stable ventricular ectopic depolarizations. A double-blind randomized study in ambulatory outpatients.

In a randomized placebo-controlled double-blind trial, we compared flecainide to quinidine for treatment of ventricular ectopic depolarizations in 19 patients. The mean percent suppression of total ventricular ectopic depolarizations was 95% for flecainide and 56% for quinidine (p less than 0.05). A greater than 80% reduction of total ventricular ectopic depolarizations was obtained in eight of nine patients given flecainide and in five of ten patients given quinidine (p = 0.09). After the randomized protocol, the patients who had received quinidine were given flecainide; 9 of the 10 patients had greater than 80% reduction of total ventricular ectopic depolarizations. Flecainide produced 100% suppression of nonsustained ventricular tachycardia and 99.5% suppression of paired ventricular depolarizations. Flecainide prolonged the PR and QRS intervals; quinidine prolonged the PR and JTC intervals. Side effects were commoner with quinidine than flecainide (p = 0.06). Three other patients failed to complete the protocol because of serious adverse experiences.

Adult↗

Endovaginal sonographic diagnosis of ectopic twin gestation.

Endovaginal sonography has permitted earlier diagnosis of ectopic pregnancy. Twin ectopic pregnancy is rare. Until now, antenatal diagnosis of twin ectopic pregnancy using endovaginal sonography had not been reported. Three cases of twin ectopic pregnancy diagnosed via the intracavitary probe are presented.

Adult↗

Gabapentin suppresses ectopic nerve discharges and reverses allodynia in neuropathic rats.

Repetitive ectopic discharges from injured afferent nerves play an important role in initiation and maintenance of neuropathic pain. Gabapentin is effective for treatment of neuropathic pain but the sites and mechanisms of its antinociceptive actions remain uncertain. In the present study, we tested a hypothesis that therapeutic doses of gabapentin suppress ectopic afferent discharge activity generated from injured peripheral nerves. Mechanical allodynia, induced by partial ligation of the sciatic nerve in rats, was determined by application of von Frey filaments to the hindpaw. Single-unit afferent nerve activity was recorded proximal to the ligated sciatic nerve site. Intravenous gabapentin, in a range of 30 to 90 mg/kg, significantly attenuated allodynia in nerve-injured rats. Furthermore, gabapentin, in the same therapeutic dose range, dose-dependently inhibited the ectopic discharge activity of 15 injured sciatic afferent nerve fibers through an action on impulse generation. However, the conduction velocity and responses of 12 normal afferent fibers to mechanical stimulation were not affected by gabapentin. Therefore, this study provides electrophysiological evidence that gabapentin is capable of suppressing the ectopic discharge activity from injured peripheral nerves. This action may contribute, at least in part, to the antiallodynic effect of gabapentin on neuropathic pain.

Acetates↗

Follicular carcinoma in ectopic thyroid gland. A case report.

Ectopic thyroid rest can be seen anywhere along the path of descent of the gland. The most ectopic thyroid tissue is a thyroglossal duct cyst associated with normal thyroid gland. Sublingual location is less common than a lingual ectopia. True malignant transformation in ectopic thyroid tissue is extremely rare. Such a malignancy is virtually always diagnosed only after surgical excision of the lesion at pathological examination. This report discusses a case of ectopic thyroid follicular carcinoma in the right submandibular region in the absence of orthotopic thyroid, discovered by chance after the surgical excision performed for a preoperative ultrasonically and cytologically misdiagnosed submandibular gland adenocystic carcinoma. The possible aetiology of such an unusual anatomical relationship is discussed as well as the importance of thyroid scanning, ultrasound and/or CT in neck lumps.

Adenocarcinoma, Follicular↗

Management of ectopic ureterocele associated with renal duplication: a comparison of partial nephrectomy and endoscopic decompression.

PURPOSE: We compared the efficacy of primary endoscopic decompression versus partial nephrectomy for treating ectopic duplex ureteroceles. MATERIALS AND METHODS: We retrospectively reviewed the records of patients with renal duplication and upper pole ectopic ureterocele. Patients were classified according to the initial radiological evaluation. The operation performed was arbitrarily chosen by the surgeon. RESULTS: A total of 54 patients had unilateral upper or bilateral upper pole ureterocele with no associated vesicoureteral reflux. Partial nephrectomy was performed in 26 patients, of whom 4 (15%) required additional surgery for new onset ipsilateral lower pole reflux. Endoscopic decompression was performed in 28 patients, of whom 18 (64%) required additional treatment due to reflux into the ipsilateral lower pole ureter and ureterocele in 9, reflux into the ureterocele only in 4, ipsilateral lower pole reflux only in 3 and persistent ureterocele obstruction in 2 (p<0.01). An ectopic ureterocele with vesicoureteral reflux into 1 or more moieties was identified in 111 patients, including 56 of 67 (84%) treated with partial nephrectomy and 37 of 44 (84%) treated with endoscopy who have persistent reflux or required further surgery for reflux resolution. CONCLUSIONS: In patients with an ectopic ureterocele and no vesicoureteral reflux partial nephrectomy should be considered the treatment of choice. However, when the initial cystogram reveals vesicoureteral reflux, partial nephrectomy and endoscopic ureterocele decompression have identical definitive cure rates of only 16%. The majority of the latter patients require continued observation and/or additional surgery for managing persistent reflux.

Abnormalities, Multiple↗

Ectopic expression of the neuropeptide pigment-dispersing factor alters behavioral rhythms in Drosophila melanogaster.

To study the function of the neuropeptide pigment-dispersing factor (PDF) in the circadian system of Drosophila, we misexpressed the pdf gene from the grasshopper Romalea in the CNS of Drosophila and investigated the effect of this on behavioral rhythmicity. pdf was either ectopically expressed in different numbers of neurons in the brain or the thoracical nervous system or overexpressed in the pacemaker neurons alone. We found severe alterations in the activity and eclosion rhythm of several but not all lines with ectopic pdf expression. Only ectopic pdf expression in neurons that projected into the dorsal central brain severely influenced activity rhythms. Therefore, we conclude that PDF acts as a neuromodulator in the dorsal central brain that is involved in the rhythmic control of behavior. Overexpression of pdf in the pacemaker neurons alone or in the other neurons that express the clock genes period (per) and timeless (tim) did not disturb the activity rhythm. Such flies still showed a rhythm in PDF accumulation in the central brain terminals. This rhythm was absent in the terminals of neurons that expressed PDF ectopically. Probably, PDF is rhythmically processed, transported, or secreted in neurons expressing per and tim, and additional PDF expression in these cells does not influence this rhythmic process. In neurons lacking per and tim, PDF appears to be continuously processed, leading to a constant PDF secretion at their nerve terminals. This may lead to conflicting signals in the rhythmic output pathway and result in a severely altered rhythmic behavior.

Animals↗

Magnetic resonance imaging of the dysplastic renal moiety and ectopic ureter.

PURPOSE: We determined the role of magnetic resonance imaging (MRI) in symptomatic children with clinically suspected and radiologically occult dysplastic renal moieties and ectopic ureters. MATERIALS AND METHODS: We reviewed clinical, imaging, cystoscopic, surgical and histological findings in 6 symptomatic children 1 to 15 years old with dysplastic renal moieties. RESULTS: After multiple conventional imaging studies failed to delineate urinary tract anatomy MRI provided detailed multiplanar images of dysplastic renal moieties that were diagnostic and predictive of subsequent intraoperative findings. Dysplastic upper pole moieties identified in 4 children were associated with ectopic ureters inserting into the vagina, prostatic urethra, bladder neck and bladder neck ureterocele in each. A solitary kidney with contralateral blind-ending ectopic ureters inserted into the bladder base in 2 cases. Pelvic cystic structures visualized by ultrasound in 3 patients were tortuous distal ureters on MRI. MRI specifically identified ureteral insertion sites that were not evident in 3 of the 5 patients who underwent cystoscopy. CONCLUSIONS: MRI may facilitate diagnosis, guide cystoscopy and aid in preoperative planning in children with poorly functioning renal moieties and ectopic ureters.

Adolescent↗

Ectopic ureterocele: results of open surgical therapy in 40 patients.

PURPOSE: The treatment of ectopic ureterocele is controversial. In addition to debate on optimal therapy, discussion exists on whether there is further risk of deteriorating bladder function after extensive bladder surgery during the first year of life, which is a reason to postpone surgery. In a prospective nonrandomized trial we treated 40 patients regardless of age who had ectopic ureterocele with complete surgical reconstruction of the lower urinary tract and upper pole resection of poorly functioning upper pole moieties at referral. Excluded from study were 3 patients with only 1 affected renal moiety initially. MATERIALS AND METHODS: We treated 31 female and 9 male patients 0 to 8.8 years old (mean age 2.17) at surgery for ectopic ureterocele extending into the bladder neck and urethra, including 19 younger than 1 year. Primary ureterocele excision was performed in 37 cases with reconstruction of the urethra, bladder neck and trigone, and ureteral reimplantation. Because of small ureterocele size, the ureterocele was left in situ in 3 patients, leading to secondary ureterocele removal due to obstructive voiding and urinary incontinence in 1 each. A staged procedure in 5 neonates involved primary lower urinary tract reconstruction with upper pole cutaneous ureterostomies followed by upper pole resection or ureteral reimplantation a few months later. After bladder neck reconstruction in 16 cases colposuspension was also done to create a normal vesicourethral angle. All patients underwent clinical and urodynamic evaluation at least 1.25 years after surgery (mean followup 5.59). Patients who were too young for the clinical assessment of continence by January 1999 were excluded from study. RESULTS: All patients are continent. A secondary endoscopic procedure was required in 13 cases, including cystoscopy only in 2, scar incision near the ureteral orifice in 3, endoscopic reflux treatment in 4, ureterocele remnant resection in 2 and bladder neck incision for obstructive voiding in 2. Secondary open bladder reconstruction was performed in another case due to a diverticulum. Postoperatively only 1 or 2 uncomplicated episodes of urinary tract infection developed in 11 patients, while there were recurrent urinary tract infections in 4. In a patient with a preexisting loss of renal function a severe infection led to renal scarring. The voiding pattern was normal in 29 patients but 11 had dysfunctional voiding, including 5 with recurrent urinary tract infection. Urodynamic followup confirmed these clinical findings. Bladder capacity in these patients was relatively high at an average of 124% of expected capacity for age. We noted no statistically significant difference in followup parameters in patients who underwent surgery before and after age 1 year. Additional colposuspension in 16 patients did not result in any significant change in outcome compared with that in patients without this procedure. CONCLUSIONS: When compared with results in the literature, complete primary lower urinary tract reconstruction in patients with ectopic ureterocele appears to have better results than a staged approach with initial endoscopic treatment. Moreover, our study provides no proof that extensive reconstructive bladder surgery in neonates and infants leads to bladder function deterioration at a later age.

Child↗

[A problem with surgical treatment of ectopic ossification after total hip replacement].

The author presents the issue and the results of surgical treatment of ectopic (heterotopic) ossification following total hip replacement. Four patients (3 female patients aged 44, 52 and 59 years and one male patient aged 58 years) in whom ectopic ossification occurred as a late complication and who had major ROM limitation were treated surgically. The three female patients were from the risk group and the evolution of ossification had a typical course, in the male patient ossification occurred 6 years after hip replacement and was preceded by trauma and a haematoma proximal to the hip prostheses. ROM limitation was more dependent on the localisation of the ossification rather than it's extent. In all cases a major increase in ROM was noted, with intra-operatively and post-op. A good functional result was found in 3 cases 12 months after surgery. In one case, where the extent of the ossification was least, ROM decreased considerably when compared to the intra-operative result. Radiological follow-up at 3, 6 and 12 months after surgical treatment didn't reveal any signs of renewal of the ossification process. Routine prophylactics of ectopic ossification consisted of administration of NSAID (Metindol 1 g/24 h) and a low-calcium diet. The author believes that cases of ectopic ossification leading to hip contractures and major ROM limitations are best treated by surgical resection.

Adult↗

[Expression of prolactin, TGF alpha and TGF beta 1 genes in estrogen-induced eutopic and ectopic pituitary prolactin-secreting tumors of rats].

The Sprague-Dawley (SD) rat bearing a heteroplasted pituitary underneath renal capsule was used to observe differential expression of prolactin (PRL), transforming growth factor alpha (TGF alpha) and transforming growth factor beta 1 (TGF beta 1) genes during the formation of pituitary prolactin-secreting tumor (prolactinoma) induced by 17 beta-estradiol (E2). Our results indicated that in both eutopic and ectopic pituitaries disconnected from hypothalamus formed simultaneously PRL-secreting tumors after the rats treated with E2 for 120 days in vivo, which was accompanied by overexpression of PRL gene (P < 0.05-0.01). The PRL mRNA level was higher in eutopic prolactinoma than that in ectopic prolactinoma (P < 0.05). Overexpression of TGF alpha and TGF beta 1 genes were also detected in eutopic prolactinoma. However, the expression of TGF alpha and TGF beta 1 genes in ectopic prolactinoma was similar to that in normal pituitary. It is suggested that TGF alpha and TGF beta 1 may be involved in prolactinoma tumorigenesis of eutopic pituitary. However, the mechanism mediating eutopic and ectopic prolactin-secreting tumor formation seems different.

Animals↗

Recurrent hyperparathyroidism after total parathyroidectomy due to multiple ectopic parathyroid glands in a patient with long-term haemodialysis.

We report the rare case of a recurrent hyperparathyroidism after total parathyreoidectomy due to multiple ectopic glands in a patient on long-term haemodialysis. In a today 47 years old man with membranoproliferative glomerulonephritis intermittent haemodialysis therapy was started in 1975. In 1982 an advanced secondary hyperparathyroidism with a parathormone (PTH) level > 500 pg/l was diagnosed; later on PTH concentration increased to 2,550 pg/ml. In 1987 total parathyroidectomy with parathyroid autograft into the left forearm was performed. After parathyroidectomy the PTH level fell to 150 pg/ml. In 1993 PTH concentration increased again to 1,750 pg/ml. There was no evidence for recurrent parathyroid glands in the neck or forearm. Therefore, we investigated the substernal region by 99mTc-tetrofosmin scintigraphy and magnetic resonance imaging. Both investigations showed evidence for two ectopic parathyroid glands in the anterior mediastinum. In June 1999 in an open thoracic surgical procedure only the greater parathyroid gland in the anterior mediastinum was isolated, but a second gland was detected in the posterior mediastinum. Both parathyroid glands were resected (histologically hyperplastic parathyroid gland tissue). After surgery the PTH level decreased to 340 pg/ml, but later on PTH increased again to > 1,000 pg/ml in January 2001. A control 99mTc-tetrofosmin scan showed evidence for a third ectopic parathyroid gland in the anterior mediastinum. Recurrent secondary hyperparathyroidism can rarely be caused by recurrent ectopic parathyroid glands in the mediastinum.

Choristoma↗

Ectopic liver lobule with portal cirrhosis.

A patient who had an ectopic liver attached to the gallbladder is reported. Angiography, scintiscanning, and routine palpation at laparotomy did not reveal the ectopic liver. Advanced liver cirrhosis was found in autopsy and the same changes were seen in the ectopic liver. The embryology, localization, symptoms and incidence of ectopic liver lobules are discussed.

Adult↗