Hyperthyroidism in early infancy: pathogenesis, clinical features and diagnosis with a focus on neonatal hyperthyroidism

We report here two rare cystic lesions, a lymphoepithelial cyst (LEC) and an epidermoid cyst of the accessory spleen (ECAS) occurring in the pancreas. Histologically, the LEC was lined by stratified squamous epithelium and surrounded by a layer of lymphoid tissue with germinal centers. The ECAS showed similar histologic features with scattered lymphoid tissue, but splenic pulp tissue was present in the wall. In both cysts, some small pancreatic islets and ducts were seen in the fibrous tissue surrounding the lymphoid layer or the splenic pulp, respectively. The lining epithelia of the LEC and the ECAS, as well as those of retention cysts of the pancreas and epidermoid cysts of the spleen used for comparison, were similarly positive with AE1/3, CAM5.2, CK7, CK13, and carcinoembryonic antigen. CA19-9 was also detected in the epithelial cells of the LEC, the ECAS, and the retention cyst of the pancreas, but not in those of the splenic epidermoid or branchial cleft cysts used for comparison. These findings indicate that LECs and ECASs might develop from the pancreatic ducts protruding into a lymph node or accessory spleen located in the pancreas, respectively. Some of both cysts might cause elevated levels of serum carcinoembryonic antigen and/or CA19-9 and should be distinguished from malignant cystic tumors.     

Total cavopulmonary anastomosis: selection criteria related to postoperative results

Cystitis cystica, a common urothelial pathology whose aetiology, morphology and clinical significance are poorly understood, affects the human urinary bladder and trigone in both sexes. We have studied the fine structure of urothelial cysts in 11 patients diagnosed cystoscopically as suffering from cystitis cystica. Several abnormal features were observed in the adjacent urothelium, including large intracellular vacuoles (4 patients), Brunn's nest (5), lymphocyte infiltration (10) and generally disorganised urothelial architecture (10). Squamous metaplasia was observed in one case. The wall of each cyst consisted of a 2-3 layered epithelium with either tall columnar or flattened cells lining the fluid-filled lumen. Both types of lining cell possessed short microvilli, while the columnar type also contained numerous membrane-bound, electron dense secretory granules in the apical cytoplasm. Rough endoplasmic reticulum, mitochondria and Golgi membranes were plentiful in the surface cells. Junctional complexes joined adjacent lining cells. The deeper cells contained relatively fewer organelles, while a basal lamina separated the cyst wall from the underlying connective tissue.     

Effect of fasting subjects'' posture on 13C-urea breath test for detection of Helicobacter pylori infection

HIV-associated salivary gland disease (HIV-SGD) includes lymphoepithelial lesions and cysts involving the salivary gland tissue and/or intraglandular lymph nodes, and Sj?gren-like conditions. Three cases of salivary gland disease occurring in HIV-infected patients are reported. Histopathological examination showed squamous epithelium-lined cysts. In the walls of the cysts lymphoid tissue, epitheloid granulomas and giant cells were found. The clinical and histopathological criteria as well as magnetic resonance imaging and therapy are discussed.     

Invasive squamous cell carcinoma arising from asymptomatic choristomatous cysts of the orbit. Two cases and a review of the literature

BACKGROUND: Epithelial choristomatous cysts are common orbital lesions, the most frequent of which are dermoid or epidermoid tumors. Massive enlargement or extraorbital extension of these benign lesions may occur. Malignant transformation of the epithelial lining of epidermoid cysts is rarely reported. METHODS: Two patients are presented in whom the epithelial lining of a previously asymptomatic choristomatous cyst of the orbit underwent malignant transformation to produce invasive squamous cell carcinoma. The unusual origin and clinical presentation of the lesions caused a delay in the diagnosis and therapy in both patients. Metastatic workup was negative in both patients, who underwent resection of their tumors via orbital exenteration with craniofacial resection. Reported cases of malignant transformation in analogous choristomatous cysts elsewhere in the cranium are reviewed. RESULTS: One patient is alive and well without recurrent disease 40 months postoperatively. The second patient died of a pulmonary embolus 2 months postoperatively. Autopsy showed no residual tumor. Overall, only 3 of 18 reported patients with epidermoid choristoma of the head and orbit with malignant transformation were alive when reported. CONCLUSIONS: Malignant squamous metaplasia is believed to be a rare complication of orbital dermoid or epidermoid cysts, with only two previously reported cases. However, malignant transformation is relatively frequent in analogous epidermoid cysts found elsewhere in the cranial vault, especially after incomplete excision. These reports encourage the complete removal of epithelial choristomas of the orbit.     

Laparoscopy for ovarian pathology in infancy and childhood

INTRODUCTION: Nonparasitic splenic cysts are a rare finding: they can be distinguished into true cysts (epidermoid or epithelial) cysts or false (pseudocysts), depending on the presence/absence of an inner epithelial lining. They are usually found during the second and third decade of life, although they can also appear during childhood. They grow bigger in children, becoming clinically apparent due to the symptoms of compression of adjacent structures. The diagnosis is radiological, made with ultrasonography (US) and computed tomography (CT). We report the US and CT patterns of splenic cysts to differentiate true from false cysts. MATERIAL AND METHODS: Our series consists of 5 patients, aged 5-17 years, all examined with US and CT; one of them was also submitted to plain abdominal radiography. Conventional CT was used in 2 cases and helical CT in the extant 3. RESULTS: All examinations yielded useful clinical informations. The only radiograph showed the rightward displacement of the gastric gas bubble US always identified the cyst and its pertinence to splenic parenchyma, although failing to assess the liquid/solid nature of a highly echogenic cyst in one case. CT confirmed the cystic nature of all masses and showed the lack of contrast enhancement of cyst walls and the presence of septa or parietal calcifications. DISCUSSION: Radiological examinations, particularly US and CT, can diagnose splenic cysts unquestionably, correctly defining the relationships with adjacent organs. CT is more sensitive than US in detecting septa or calcifications, which are definitely useful findings to distinguish true from false cysts, since internal septa are more frequent in true cysts while parietal calcifications are typical of pseudocysts. The final diagnosis, however, is made at histology.     

Observations on two cases of apparent submandibular gland cysts in HIV positive patients: MR and CT findings

OBJECTIVE: To present two cases of probable lymphoepithelial cysts of the submandibular glands in patients who were human immunodeficiency virus (HIV) positive and who also had lymphoepithelial cysts of the parotid glands. MATERIALS AND METHODS: Computed tomography and MRI of two HIV positive patients with lymphoepithelial cysts of the parotid glands and cysts in the submandibular glands were correlated with the histories and the possible presence of other known causes of submandibular gland multiple cysts. RESULTS: Because of the present treatment philosophy regarding HIV positive patients with major salivary gland cysts, surgical resection of these glands was not performed. All other known causes of multiple submandibular gland cysts were excluded by either history or laboratory data. CONCLUSION: Computed tomography and MRI on two patients with known HIV infection and bilateral parotid lymphoepithelial cysts are presented. Both patients also had bilateral multiple submandibular gland cysts and no evidence of obstructive glandular disease, autoimmune disease, or other organ system cysts. These cases of presumed submandibular gland lymphoepithelial cysts are rare in the literature. They are presented in the hope that other radiologists will be stimulated to document the occurrence of this entity.     

Underreporting of energy intake in 1 to 18 year old German children and adolescents

AIMS: Intraosseous jaw cysts with a solely orthokeratinized lining epithelium have been suggested to differ from the typical odontogenic keratocysts (OKC) by exhibiting a less aggressive behaviour. We report 15 cases of such cyst type under the term of 'orthokeratinized odontogenic cyst (OOC)' and compared their clinical, histological and immunocytochemical features with that of OKC. METHODS AND RESULTS: The cysts of the present series were all solitary lesions, occurred mostly in young male patients, and showed a predilection for the posterior mandible areas. Follow-up of 14 patients, nine of whom were treated by simple enucleation, revealed no recurrence over a period of 3.5-12 years after surgery. None of the patients had any association with the naevoid basal cell carcinoma syndrome. Furthermore, histological and immunocytochemical comparison between OOC and OKC revealed marked differences in their morphology and epithelial expression. The lining epithelium of OOC lacked the typical features of OKC and appeared to show a lower proliferative activity. CONCLUSION: These findings suggest that OOC is clinicopathologically separate from other types of odontogenic cysts and may thus constitute a distinct clinical entity.     

Migration resistant, blood-compatible plasticized polyvinyl chloride for medical and related applications

AIMS: To determine the morphology, immunophenotype and bcl-2 protein status of intraepithelial lymphocytes in HIV-positive lymphoepithelial lesions. METHODS AND RESULTS: Seventeen cases (from adults and children) of HIV-associated parotid and lung lymphoid lesions were examined. In addition, three lymphoepithelial cysts from HIV-negative patients were studied in parallel. Immunohistochemistry was performed on paraffin embedded tissue with the following antibodies: CD20, CD79a, CD3, CD4, CD8, bcl-2, CAM5.2, AE1/3, MIB1, kappa/lambda light chains and EBV-LMP-1. Heavy chain rearrangement was sought by polymerase chain reaction (PCR) in four of the cases. The lymphocytes participating in lymphoepithelial lesions of HIV-positive patients had the morphology of centrocyte-like cells with occasional cells resembling centroblasts. The majority of these cells were of B-cell lineage, but occasional intraepithelial T-cells (CD8 positive, CD4 negative) were also present. T-cells also formed a significant component of the infiltrative lymphoid cells outside the lymphoepithelial lesions. These were mainly CD8 positive, but very occasional CD4-positive T-cells were also noted. None of the cases showed light chain restriction and the four cases did not demonstrate heavy chain rearrangement by molecular biology. The interesting finding was the absence of bcl-2 expression by the intraepithelial lymphocytes. In contrast, the intraepithelial lymphocytes seen in the non-HIV setting were strongly bcl-2 positive. The majority of these were B-cells, and very occasional CD8 and CD4 positive T-cells formed the intraepithelial population. CONCLUSION: It is postulated that this finding is due to the HIV causing down-regulation of bcl-2 protein.     

Severe calcifying atherosclerosis of the thoracic aorta with symptoms of aortic isthmus stenosis. Case report

Mesenteric cysts are relatively rare tumors, and most patients present nonspecific abdominal symptoms until developing larger cysts. We report two cases of mesenteric cysts that were totally excised during laparoscopic surgery. The cysts of both patients were located in the mesenterium of the cecum or ascending colon. Under laparoscopy, the cyst was punctured to collect the cystic fluid for cytology and then completely removed without bowel resection. Histological examination revealed cystic lymphangiomas with endothelial cell lining. The postoperative course was uneventful. Laparoscopic treatment of benign abdominal cysts is an alternative safe and less invasive operation.     

Cystic adventitial degeneration of the popliteal artery. Rare cause of intermittent claudication in middle-aged adults

We report on a 36-year old male patient with clinically prolonged intermittent claudication of the right leg. An operative specimen measuring 8.0 cm of the A. poplitea was sent for examination, which was sheathed by an aneurysmal cyst having a maximal diameter of 1.5 cm. Examination under the light microscope showed an incomplete septated ganglion-like cystic formation with flat connective tissue-like cells or even larger histiocytoid cell elements as inner lining. Immunohistochemically, the cells on the inner lining of the cyst showed to be strongly positive to macrophage marker PG-M1, KP1 and Ki-Mp1. The endothelium of the vessels of the wall of the cyst showed a marked expression of cytokeratin 18. Immunohistochemically as well as in conventional histology, there are general parallels between cysts in cystic adventitial degeneration, ganglia and normal synovia as the two last mentioned tissues also show an expression of cytokeratin 18 at the endothelia of smaller vessels as well as an expression of macrophage-associated antigens on cells of the inner lining. Aetiopathologically, the cysts developing in the course of the disease are rather adventitial ganglia.     

Immunolocalization of interstitial collagenase (MMP-1) and tissue inhibitor of metalloproteinases-1 (TIMP-1) in radicular cysts

To investigate the mechanisms involved in expansion of radicular cysts, monoclonal antibodies against interstitial collagenase (MMP-1) and tissue inhibitor of metalloproteinases-1 (TIMP-1) were used to localize the sites of MMP-1 and TIMP-1 expression in 30 radicular cysts. Positive MMP-1 staining was detected in the lining epithelium and subepithelial fibroblasts, macrophages, endothelial cells and osteoblasts/osteocytes in all specimens. Positive TIMP-1 staining was identified in osteoblasts/osteocytes and endothelial cells of all specimens, and in the lining epithelium and subepithelial fibrous connective tissue wall of five radicular cysts with an intense inflammatory cell infiltrate. The number and distribution of positive cells for MMP-1 or TIMP-1 varied widely among individual specimens, but strong immunostaining was constantly detected at sites with prominent subepithelial inflammation. Results here support the hypothesis that MMP-1 may play an important role in the expansion of radicular cysts. The absence of TIMP-1 expression in lining epithelium and subepithelial fibroblasts and macrophages in most cases studied indicated that an imbalance between MMP-1 and TIMP-1 production may lead to radicular cyst expansion.     

Cutaneous necrosis associated with acquired severe protein S deficiency

The incidence of benign lymphoepithelial lesions of the parotid gland in patients afflicted with HIV has increased. Enucleation is a safe and effective procedure that provides the patient with complete removal of the cyst and a low recurrence rate. Enucleation of these parotid cysts should be considered in treating these patients.     

Ultrasonography of cystic thyroid nodules: sonographic-pathologic correlation

Retrospectively the ultrasonographic findings of 153 surgically resected cystic thyroid nodules were reviewed. The pathologic findings in this series revealed that 86% were degenerating benign adenomas or adenomatous goiters, and 14% were malignant tumors. The sonographic appearance of these lesions was classified into 7 groups as follows: type I: entirely cystic (less than 1cm), type II: cystic(more than 1cm) [II(a)], and with small polyp or dome-like elevation on the cyst wall [II(b)], type III: larger cyst with projection (more than 1cm) into the lumen, type IV: cyst with a peripherally localized solid component, type V: irregularly mixed cystic and solid components, type VI: a solid mass with multiple crescentic cysts [VI(a)], or round cysts [VI(b)], type VII: a solid mass with only one or two cysts. Pathologic correlation revealed that malignancy in this series ranged from 80% in type III and V to only 4% in type II, where most of the lesions in this group were composed of granulation tissue in degenerating adenomatous polyps and cyst walls. Lesions in type IV showed malignancy rate of 40%. Type III showed characteristic sonographic findings seen in cystic papillary carcinomas (CPCs), with multiple punctate echogenic foci in large pedunculated projections. The typical psammomatous calcifications specific in this group were confirmed in 6 of the 8 type III CPCs. The multiple crescentic cysts in type VI(a) lesions were characteristic sonographic signs seen in adenomatous goiters, representing the pathologic finding of cysts forming around each of multiple adenomatous nodules in this group. Type VII represented non specific appearing lesions, included adenomas, adenomatous goiters, CPCs and follicular carcinomas.(ABSTRACT TRUNCATED AT 250 WORDS)     

Failure in self care and heart failure, thiamine deficiency in geriatric patients

The ultrasonographic findings of 25 lesions in 23 patients with surgically proven ovarian masses were reviewed. There were 10 cystic teratomas, two simple cysts, two follicular cysts, two mucinous cystadenomas, two NHL, one corpus luteum cyst, one hydrosalpinx, one serous cystadenoma, one yolk sac carcinoma, one dysgerminoma, one embryonal carcinoma, and one mixed form (yolk sac carcinoma, choriocarcinoma). All patients were less than 15 years old. We classified all cases into four patterns: cystic, cystic with mural nodule, mixed, and solid. Eight lesions of the cystic pattern included two simple cysts, two follicular, cysts and one corpus luteum cyst. The other lesions were benign, too. Nine lesions with the cystic with mural nodule pattern consisted of eight cystic teratomas and one mucinous cystadenoma. All lesions were benign. The mixed pattern was seen in four lesions, half of which were malignant, i. e., one embryonal carcinoma and one yolk sac carcinoma. Four lesions with the solid pattern were all malignant masses: one dysgerminoma, two NHL and one mixed form. In this classification, the cystic and cystic with mural nodule patterns are benign, while mixed and solid patterns are highly suggestive of malignancy.     

Investigations on the distribution of HBs antigen subtypes

The localisations of bone cysts known under the title of "intraosseous ganglion, synovial cyst of bone and solitary unicameral cyst" are summarised from the literature. Two of the authors' patients are added to the few cases of talar cysts recorded up to the present. In addition, a patient with a calcaneal cyst is described. The cysts can be traumatic or non-traumatic in origin. The various theories concerning their etiology are discussed. The clinical findings, differential diagnosis and therapy of subchondral bone cysts particularly in the region of the foot are considered.     

Cystic thyroid lesions in children

BACKGROUND/PURPOSE: Cystic lesions of the thyroid encompass a wide and heterogeneous group of disease states in children, ranging from benign purely cystic entities to malignant tumors. The purpose of this study was to study both the presentation and management of cystic thyroid lesions in the pediatric population. METHODS: A retrospective review of all thyroid masses presenting between 1978 and 1996 and found to be purely or partially cystic on ultrasound examination was conducted, looking at presentation, family history, laboratory values, ultrasound scan and radionuclide imaging, and pathological and cytological evaluation. RESULTS: Twenty-four patients (19 girls, 5 boys) aged 6 to 18 years received the diagnosis of cystic lesions of the thyroid. Of these, 23 presented with painless neck masses, 21 were clinically euthyroid, only one had a single abnormal thyroid function test, only two had mildly positive antithyroid antibody test results, and nearly 30% had a positive family history of thyroid disease. Ultrasonography showed pure cysts in five patients and mixed solid cystic lesions in 19 patients. On scintiscan, six lesions were hot, 13 were cold, three showed normal uptake, and two were mixed. Treatment included either observation, aspiration, cyst sclerosis, surgery, or combinations thereof. Pathological and cytological results included follicular adenoma (n = 9), cystic degeneration (n = 6), multinodular goiter (n = 4), carcinoma (n = 2), branchial cleft cyst (n = 1), and undetermined (n = 2). CONCLUSIONS: Thyroid cysts are often thought to represent benign degenerative disease. Our study, which is the first in the literature to specifically address thyroid cysts in children, shows that ultrasound scan is useful in evaluating thyroid masses, whereas laboratory and radionuclide are of less value, and that single lesions of mixed echogeneity are likely to represent neoplasms, a significant percentage of which are malignant.     

The clinical neuropsychological aspects in the surgical treatment of arteriovenous malformations of the gyrus cinguli

The dependence of the tumor cells differentiation upon the tumor histoarchitectonics, tumor type and patients' sex is studied immunohistochemically and by the in situ hybridization in 57 cases of thyroid carcinoma in children from the Belarus republic. A significant decrease of thyroglobulin synthesis and accumulation in the solid structures of papillary carcinoma is found. A decrease of the degree of the tumor biological differentiation in males is observed regardless of tumor histoarchitectonics. Increased accumulation of thyroglobulin (without change in its synthesis) in normal follicular epithelium found in parallel with increased intrathyroidal carcinoma dissemination and the size of the dominant tumor needs further investigation.     

Evaluation of the obstetrical risks of the antiphospholipid syndrome and therapeutic management

In experiments using ferric nitrilotriacetate (Fe-NTA) as a renal carcinogen, multiple renal cysts are often observed in addition to renal tumours. In the present study, we used 3-week-old male ddY mice and examined the relation between renal cysts and cancer development. Four months after the start of Fe-NTA administration, we observed cysts in the renal cortex in all Fe-NTA-treated mice, but not in Fe-free NTA-treated mice. Three types of cysts were observed, but only those which originated from the renal proximal tubules showed multilayered or papillary growth of cyst epithelial cells. Using histochemical staining, we found a cyst formation-tumour induction sequence, and the supposed cystic-papillary tumour induced by Fe-NTA was of proximal tubular cell origin. We also found that the minimum dose of Fe-NTA capable of inducing renal tumours in ddY mice was 10 mg of iron/kg/day, four times in 2 weeks.     

Orbital dermoid cysts: clinicopathologic correlations, classification, and management. The 1997 Josephine E. Schueler Lecture

The dermoid cyst is one of the most common space-occupying orbital lesions, but there has been no large series that details the clinical, pathologic, and therapeutic aspects of this condition. Furthermore, despite the various presentations and types of dermoid cysts, there is no clinically applicable classification of these lesions. We performed a retrospective histopathologic study of 197 consecutive orbital and periorbital dermoid cysts excised at Wills Eye Hospital during a 32-year period, correlated the histopathologic findings with the recorded clinical and radiographic observations, and devised a clinically applicable classification of orbital dermoid cysts. The mean age at excision was 17 years (range, 5 months to 85 years), and the most frequent clinical finding was a painless subcutaneous mass, present in 89% of cases. In 72% of patients, the mass was located in the superotemporal orbital region and produced no abnormalities of vision or intraocular pressure. Computed tomography and magnetic resonance imaging, performed mainly in the more recent cases, disclosed a characteristic round to ovoid cystic lesion with a well-defined lining. Histopathologically, 165 of the cysts (84%) were lined by keratinizing, stratified squamous epithelium. Ten (5%) were lined by nonkeratinizing epithelium resembling conjunctival epithelium. Hair shafts were present in the wall or lumen of the cyst in 195 (99%) of specimens, sebaceous glands in 148 (75%), and sweat glands in 39 (20%); inflammation was present in 75 cases (38%). Based on these personal observations and a review of the literature, a classification of orbital dermoid cysts is proposed that we believe will provide guidance for the ophthalmologist in the management of these lesions.