scholarly journals OTHR-14. DIENCEPHALIC SYNDROME SECONDARY TO PITUITARY STALK THICKENING

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii424-iii424
Author(s):  
Carlos Almeida ◽  
Bruna Minniti Mançano ◽  
Gilda D’Agostino Eugui ◽  
Marcus Matsushita ◽  
Gabrielle Alvarenga ◽  
...  
Keyword(s):  
Histopathological Examination ◽  
Caloric Intake ◽  
Rare Condition ◽  
Pituitary Stalk ◽  
Hormone Deficiency ◽  
Transverse Diameter ◽  
Diagnostic Challenge ◽  
Suprasellar Region ◽  
Diencephalic Syndrome ◽  
Pituitary Stalk Thickening

Abstract BACKGROUND Diencephalic syndrome (DS) is a rare condition associated with neoplastic lesions of the sellar-suprasellar region, whose pathophysiological mechanisms are still unclear. DS occurs in <10% of hypothalamic gliomas and has also been described in suprasellar germinomas, craniopharyngiomas, epidermoid cysts, rarely with non-suprasellar lesions such as brainstem gliomas. DS has not been associated with isolated pituitary stalk thickening. Isolated pituitary stalk thickening (IPST) presents a diagnostic challenge, ranging from benign (craniopharyngioma) to malignant lesions (germinoma, metastasis, histiocytoses of the Langerhans group). The coexistence of diabetes insipidus (DI) with anterior pituitary dysfunction and IPST implies more risk to harbor neoplasia. CASE REPORT: A 6-year old girl presented with DI and inadequate weight gain (despite regular caloric intake) and preservation of linear growth. Neurological examination showed no abnormalities. However, physical examination revealed a malnourished patient (both weight-for-age value and body-mass-index below the third percentile). Blood tests and negative IgA anti-endomysial antibodies excluded malabsorption as a cause of her malnutrition; endocrine work-up excluded thyroid dysfunction, growth hormone deficiency, and adrenal insufficiency. Magnetic resonance imaging (MRI) showed thickening of the pituitary stalk with a transverse diameter of 7 mm. The patient underwent a biopsy through a supraorbital eyebrow approach. Histopathological examination revealed lymphocytic hypophysitis, with tissue markers all negative for germinoma. The girl is currently under follow up with serial MRI every three months. CONCLUSION DS should be considered as a differential diagnosis in any child with failure to thrive, and imaging studies should be performed even if there are no additional neurological symptoms.

scholarly journals Evolution of childhood central diabetes insipidus into panhypopituitarism with a large hypothalamic mass: is 'lymphocytic infundibuloneurohypophysitis' in children a different entity?

1998 ◽  
Vol 139 (6) ◽  
pp. 635-640 ◽  
Author(s):  
M Maghnie ◽  
E Genovese ◽  
MG Sommaruga ◽  
M Arico ◽  
D Locatelli ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Diabetes Insipidus ◽  
Histopathological Examination ◽  
Dynamic Magnetic Resonance Imaging ◽  
Central Diabetes Insipidus ◽  
Pituitary Stalk ◽  
Hormone Deficiency ◽  
Resonance Imaging ◽  
Dynamic Magnetic Resonance

We report on a 15-year-old girl who had presented with acute onset central diabetes insipidus at the age of 8 years; this was followed by growth failure due to acquired growth hormone deficiency. Initial magnetic resonance imaging showed a uniformly enlarged pituitary stalk and absence of posterior pituitary hyperintensity. Frequent patient examination and magnetic resonance imaging gave unchanged results until after 5 years a large hypothalamic mass and panhypopituitarism were found. Dynamic magnetic resonance imaging documented hypothalamic-pituitary vasculopathy. Histopathological examination revealed perivascular inflammatory lymphoplasmic infiltrates with no granulomatosis or necrosis and negative staining for S-100 protein, suggesting autoimmune inflammatory disease (lymphocytic infundibuloneurohypophysitis?). The response to glucocorticoid pulses (30 mg/kg per day for 3 days i.v.) was favorable. the hypothalamic mass being halved and partial anterior pituitary function recovery maintained for 2 years after the start of treatment. We suggest that long-term surveillance is needed for isolated and chronic thickening of the pituitary stalk and that dynamic magnetic resonance imaging can contribute to the demonstration of hypothalamic-pituitary vascular impairment associated with local vasculitis.

Neoplastic Etiology and Natural Course of Pituitary Stalk Thickening: A Systematic Review and Meta-analysis

2021 ◽  
Author(s):  
Dong Yeong Kim ◽  
Pyeong Hwa Kim ◽  
Ah Young Jung ◽  
Jin-Ho Choi ◽  
Young Ah Cho ◽  
...  
Keyword(s):  
Meta Analysis ◽  
Natural Course ◽  
Pituitary Stalk ◽  
Hormone Deficiency ◽  
Original Research ◽  
Pituitary Hormone ◽  
Pediatric Populations ◽  
The Difference ◽  
Pituitary Stalk Thickening

Abstract Context Pituitary stalk thickening (PST) is often identified on magnetic resonance imaging (MRI), either incidentally or during diagnostic work-up of hypopituitarism. However, the neoplastic etiology and natural course of PST are not fully understood, although this knowledge is required to establish diagnostic and surveillance strategies. Objectives To investigate the neoplastic etiology and natural course of PST. Methods MEDLINE/PubMed and EMBASE databases were searched up to February 2021 to identify original research investigating the etiologies of PST. The proportion of neoplastic etiology in patients with PST was meta-analytically pooled. Supplementary analysis exploring factors suggesting neoplasm was also performed. For initially indeterminate cases without confirmed diagnosis, the proportion of patients showing progression of PST during follow-up was evaluated. Results Eighteen studies covering 1368 patients with PST were included. The pooled proportion of neoplasm was 45.2% (95% CI, 33.3–57.8%), with substantial heterogeneity across studies (I 2=93%). The most common neoplasm was germ cell tumor (14.0% of study population), followed by Langerhans cell histiocytosis (10.2%) and metastasis (4.7%). The studies on pediatric populations and those with >50% of patients having at least one pituitary hormone deficiency tended to show a higher proportion of neoplasm. The pituitary stalk was thicker in neoplasms, but the difference was not significant (pooled mean difference, 2.08 mm; P=0.08). In initially indeterminate cases, 18.5% (95% CI, 7.6–38.3%) showed progression of PST during follow-up. Conclusion PST was commonly confirmed to be neoplastic, especially in pediatric populations. As isolated PST frequently progresses, follow-up imaging is essential in initially indeterminate cases.

Autoimmune Pancreatitis – Diagnosis, Management and Longterm Follow-up

2014 ◽  
Vol 23 (2) ◽  
pp. 179-185 ◽  
Author(s):  
Suvadip Chatterjee ◽  
Kofi W. Oppong ◽  
John S. Scott ◽  
Dave E. Jones ◽  
Richard M. Charnley ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Rare Condition ◽  
Team Approach ◽  
Diagnostic Challenge ◽  
Multisystem Disorder ◽  
North East ◽  
The North ◽  
Work Up ◽  
Igg4 Level

Background & Aims: Autoimmune pancreatitis (AIP) is a fibroinflammatory condition affecting the pancreas and could present as a multisystem disorder. Diagnosis and management can pose a diagnostic challenge in certain groups of patients. We report our experience of managing this condition in a tertiary pancreaticobiliary centre in the North East of England.Methods: Patients were identified from a prospectively maintained database of patients diagnosed with AIP between 2005 and 2013. Diagnosis of definite/probable AIP was based on the revised HISORt criteria. When indicated, patients were treated with steroids and relapses were treated with azathioprine. All patients have been followed up to date.Results: Twenty-two patients were diagnosed with AIP during this period. All patients had pancreatic protocol CT performed while some patients had either MR or EUS as part of the work up. Fourteen out of 22 (64%) had an elevated IgG4 level (mean: 10.9 g/L; range 3.4 - 31 g/L). Four (18%) patients underwent surgery. Extrapancreatic involvement was seen in 15 (68%) patients, with biliary involvement being the commonest. Nineteen (86%) were treated with steroids and five (23%) required further immunosuppression for treatment of relapses. The mean follow up period was 36.94 months (range 7 - 94).Conclusion: Autoimmune pancreatitis is being increasingly recognized in the British population. Extrapancreatic involvement, particularly extrahepatic biliary involvement seems to be a frequent feature.Diagnosis should be based on accepted criteria as this significantly reduces the chances of overlooking malignancy. Awareness of this relatively rare condition and a multi-disciplinary team approach will help us to diagnose and treat this condition more efiectively thereby reducing unnecessary interventions.

scholarly journals Fetus in fetu: two case reports from North African country

2021 ◽  
Vol 69 (1) ◽  
Author(s):  
Moutaz Ragab ◽  
Omar Nagy Abdelhakeem ◽  
Omar Mansour ◽  
Mai Gad ◽  
Hesham Anwar Hussein
Keyword(s):  
Histopathological Examination ◽  
Case Reports ◽  
Abdominal Mass ◽  
Mature Teratoma ◽  
Surgical Techniques ◽  
Rare Condition ◽  
Mass Effect ◽  
Fetus In Fetu ◽  
Vascular Connection ◽  
First Case

Abstract Background Fetus in fetu is a rare congenital anomaly. The exact etiology is unclear; one of the mostly accepted theories is the occurrence of an embryological insult occurring in a diamniotic monochorionic twin leading to asymmetrical division of the blastocyst mass. Commonly, they present in the infancy with clinical picture related to their mass effect. About 80% of cases are in the abdomen retroperitoneally. Case presentation We present two cases of this rare condition. The first case was for a 10-year-old girl that presented with anemia and abdominal mass, while the second case was for a 4-month-old boy that was diagnosed antenatally by ultrasound. Both cases had vertebrae, recognizable fetal organs, and skin coverage. Both had a distinct sac. The second case had a vascular connection with the host arising from the superior mesenteric artery. Both cases were intra-abdominal and showed normal levels of alpha-fetoprotein. Histopathological examination revealed elements from the three germ layers without any evidence of immature cells ruling out teratoma as a differential diagnosis. Conclusions Owing to its rarity, fetus in fetu requires a high degree of suspicion and meticulous surgical techniques to avoid either injury of the adjacent vital structures or bleeding from the main blood supply connection to the host. It should be differentiated from mature teratoma.

scholarly journals AB0945 CONTRIBUTION OF MICROBIOLOGICAL AND ANATOMOPATHOLOGICAL EXAMINATIONS IN THE DIAGNOSIS OF SPONTANEOUS PYOGENIC SPONDYLODISCITIS IN ADULTS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1771.3-1771
Author(s):  
I. Mahmoud ◽  
M. Moalla ◽  
A. Ben Tekaya ◽  
S. Bouden ◽  
R. Tekaya ◽  
...  
Keyword(s):  
Histopathological Examination ◽  
Klebsiella Oxytoca ◽  
Rare Condition ◽  
Blood Cultures ◽  
X Rays ◽  
Skin Sample ◽  
Bacteriological Study ◽  
Non Invasive ◽  
Pyogenic Spondylodiscitis ◽  
Serious Infection

Background:Pyogenic spondylodiscitis (SPD) is a serious infection of an intervertebral disc and/or adjacent vertebrae, that remains a topical problem in rheumatological practice. Early diagnosis and treatment are the only guarantees of a favorable outcome. Clinicians must strive to isolate the responsible bacteria in order to adapt the treatment, and thus reduce the risk of resistance and complications due to SPD itself, but also to the multiplication of probabilistic treatments.Objectives:Our aim was to study the contribution of the different microbiological and anatomopathological examinations in the diagnosis of pyogenic SPD.Methods:It was a descriptive study in a single rheumatology department. Data were collected retrospectively from observations of patients hospitalized in the past 20 years who have been diagnosed with pyogenic SPD. We excluded cases of tuberculous and brucellar SPD from our study because of their completely different histological and microbiological profiles.Results:Twenty-two cases of pyogenic SPD were collected (14M/ 8F). The mean age of the population was 55.9 years [29,80]. A bacteriological survey including at least one cytobacteriological examination of the urine (CBEU), chest X-rays and blood cultures allowed the identification of the bacteria in 16 cases (73%). The most common site were bacteria was identified was blood culture in 7 cases, skin sample and urine collection in 2 cases each. Disco-vertebral puncture and biopsy (DVPB) was performed in 19 patients when there was no bacteria identification and/or when diagnosis of infectious SPD persisted doubtful. On histopathological examination, were described: an infiltrate and/or inflammatory changes without specificity signs in 7 patients and an appearance of chronic pyogenic SPD very likely in 12 patients. Bacteriological study of DVPB fluid or paravertebral abscesses sample helped to isolate bacteria in 4 patients. DVPB or abscesses puncture were contributing by histological and/or bacteriological examination in 12 patients (63%).Infecting bacteria was identified in 14 patients (64%). Gram-negative bacilli (GNB) and staphylococcus aureus were the most frequent germs (7 cases each) including 2 cases of co-infection. GNBs were represented by: Escherichia Coli and Enterobacter Cloacae in 2 cases each, Proteus Mirabilis, Serratia Marcescens and Klebsiella oxytoca in 1 case each. Clostridium clostridioforme and Lactococcus cremoris were isolated in 1 case each. For patients whose etiological investigation remained negative, SPD diagnosis was retained based on imaging (MRI) guided by anamnestic, clinico-biological and histopathological arguments.Conclusion:SPD is a rare condition that needs to be treated rapidly. Once the diagnosis is suspected, bacteria must be isolated before starting any antibiotic therapy. Simple and non-invasive exams as blood cultures, CBUE and chest rays, should be undertaken first. In fact, these simple exams allowed a germ identification in 73% cases in our study. If doubt persist, DVPB could be contributive to the diagnosis.References:NoneDisclosure of Interests:None declared

scholarly journals An intrasellar germinoma with normal tumor marker concentrations mimicking primary lymphocytic hypophysitis

2013 ◽  
Vol 57 (7) ◽  
pp. 566-570 ◽  
Author(s):  
Mariana F. Guzzo ◽  
Cristina B. Formiga Bueno ◽  
Thiago T. Amancio ◽  
Sergio Rosemberg ◽  
Cleonice Bueno ◽  
...  
Keyword(s):  
Clinical Laboratory ◽  
Correct Diagnosis ◽  
Careful Analysis ◽  
Malignant Neoplasms ◽  
Pituitary Hormone ◽  
Appropriate Treatment ◽  
Suprasellar Region ◽  
Neoplastic Lesions ◽  
Posterior Pituitary Hormone ◽  
Pituitary Stalk Thickening

Intracranial germinomas (GE) are malignant neoplasms most commonly found in the suprasellar region, which may cause anterior and particularly posterior pituitary hormone deficits with central diabetes insipidus (DI). Differential diagnosis of pituitary stalk thickening includes granulomatous, inflammatory, infectious, and neoplastic lesions. Although careful analysis of clinical, laboratory, and imaging findings may facilitate the diagnosis, transsphenoidal biopsy is indicated to confirm the disease, as the correct diagnosis directs the appropriate treatment.

Struma Ovarii: Clinico-Morphological Features and Therapeutic Experience of a Romanian Institution over 20 Years

2021 ◽  
Vol 11 (20) ◽  
pp. 9427
Author(s):  
Mihaela Camelia Tîrnovanu ◽  
Vlad Gabriel Tîrnovanu ◽  
Bogdan Florin Toma ◽  
Elena Cojocaru ◽  
Carmen Ungureanu ◽  
...  
Keyword(s):  
Rare Condition ◽  
Diagnostic Algorithm ◽  
Complete Information ◽  
Published Data ◽  
Struma Ovarii ◽  
Therapeutic Approaches ◽  
Diagnostic Challenge ◽  
Benign Ovarian Tumors ◽  
The Right ◽  
Clinical Conditions

Struma ovarii is a rare condition with scarce published data regarding clinical, morphological, and therapeutic approaches. This study reports the experience of 25 patients with struma ovarii who received surgical treatment in a gynecology department in Romania. The study was conducted from January 1999 to September 2021 and included patients with confirmed struma ovarii whose medical records were retrospectively reviewed and evaluated. Struma ovarii represented 2.8% of the total number of benign ovarian tumors treated by surgery. The age of the patients was between 24 and 71 years. The tumor was unilateral in 24 cases, 13 cases on the left ovary, 11 on the right side, and bilateral in 1 case. Tumor dimensions ranged between 1 cm and 20 cm. In two cases, the patients had symptoms of hyperthyroidism. The procedure was performed on four women for diagnoses other than an ovarian tumor. In another five situations, there was suspicion of ovarian malignancy. In addition, struma ovarii was associated with other clinical conditions in 22 cases. These lesions represent a diagnostic challenge with heterogeneous clinical and imaging manifestations. Complete information of clinical, morphologic, and surgical findings may improve the diagnostic algorithm and better predict patient outcomes.

scholarly journals Tubulocystic renal cell carcinoma: Report of a rare case

2015 ◽  
Vol 9 (9-10) ◽  
pp. 654 ◽  
Author(s):  
Aanchal Kakkar ◽  
Mehar C. Sharma ◽  
Manpreet Uppal ◽  
Sunil Chumber
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Histopathological Examination ◽  
Cystic Neoplasm ◽  
Diagnostic Challenge ◽  
Elderly Male ◽  
Diagnostic Dilemma ◽  
Cystic Neoplasms ◽  
Atypical Cells

Cystic neoplasms of the kidney are rare, and present a unique diagnostic challenge. We report the case of an elderly male who presented with a large cystic neoplasm, which was a diagnostic dilemma clinically and radiologically. Histopathological examination showed a tumour composed of variably sized tubules lined by atypical cells having large round nuclei with prominent nucleoli. Hobnailing was seen at places. Tumour cells were immunopositive for pancytokeratin, vimentin, CD10, CK19 and AMACR, confirming a diagnosis of tubulocystic renal cell carcinoma (TC-RCC).

scholarly journals Pituitary Morphology and Function in 43 Children with Central Diabetes Insipidus

2016 ◽  
Vol 2016 ◽  
pp. 1-7 ◽  
Author(s):  
Wendong Liu ◽  
Limin Wang ◽  
Minghua Liu ◽  
Guimei Li
Keyword(s):  
Diabetes Insipidus ◽  
Central Diabetes Insipidus ◽  
Pituitary Function ◽  
Pituitary Stalk ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Initial Manifestation ◽  
Stalk Diameter ◽  
And Function

Objective. In pediatric central diabetes insipidus (CDI), etiology diagnosis and pituitary function monitoring are usually delayed. This study aimed to illustrate the importance of regular follow-up and pituitary function monitoring in pediatric CDI.Methods. The clinical, hormonal, and neuroradiological characteristics of children with CDI at diagnosis and during 1.5–2-year follow-up were collected and analyzed.Results. The study included 43 CDI patients. The mean interval between initial manifestation and diagnosis was 22.29 ± 3.67 months (range: 2–108 months). The most common complaint was polyuria/polydipsia. Causes included Langerhans cell histiocytosis, germinoma, and craniopharyngioma in 2, 5, and 4 patients; the remaining were idiopathic. No significant changes were found during the 1.5–2 years after CDI diagnosis. Twenty-three of the 43 cases (53.5%) had ≥1 anterior pituitary hormone deficiency. Isolated growth hormone deficiency was the most frequent abnormality (37.5%) and was not associated with pituitary stalk diameter. Multiple pituitary hormone deficiencies were found in 8 cases with pituitary stalk diameter > 4.5 mm.Conclusion. Diagnosis of CDI is usually delayed. CDI with a pituitary stalk diameter > 4.5 mm carries a higher risk of multiple pituitary hormone deficiencies. Long-term MRI and pituitary function follow-ups are necessary for children with idiopathic CDI.

scholarly journals The Frequency and Causes of Subepidermal Gaps or Blisters and the Compliance Rate Between Clinical Diagnosis and Pathology Reports from 2015 to 2019

2021 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Abbas Darjani ◽  
Hojat Eftekhari ◽  
Seyedeh Rojin Amini Rad ◽  
Narges Alizadeh ◽  
Rana Rafiee ◽  
...  
Keyword(s):  
Clinical Diagnosis ◽  
Skin Diseases ◽  
Histopathological Examination ◽  
Compliance Rate ◽  
Clinical Manifestations ◽  
Final Diagnosis ◽  
Direct Immunofluorescence ◽  
Diagnostic Challenge ◽  
Cross Sectional ◽  
Pathology Reports

Background: Skin diseases are the fourth most common cause of human illness, and blisters with different clinical manifestations make a diagnostic challenge. Objectives: This study aimed to evaluate the frequency and causes of subepidermal gaps or blisters, as well as the compliance rate between the initial and final clinical diagnoses based on pathology reports. Methods: In this cross-sectional study, pathology reports of subepidermal blisters or gaps were evaluated in the patients referred to the Razi Laboratory of Rasht from 2015 to 2019. The samples were examined by a pathologist after hematoxylin and eosin staining. The reports included demographic information, clinical differential diagnoses, final diagnosis, direct immunofluorescence findings, and salt split results. Finally, the compliance rate of clinical diagnosis with pathology reports was determined. Results: A total of 183 pathology reports were evaluated, 170 of which contained the final diagnosis. Females were more frequently affected by the disease, and pemphigoid bolus and lichen planus were the most prevalent final diagnoses. The compliance rate between the initial and final diagnoses was 94%. About 37.2% of the reports lacked direct immunofluorescence (DIF) and salt split, and only 42.6% of the samples had undergone DIF examination, while 20.2% had both DIF and salt split. There was no significant association between the compliance rate of the final diagnosis with age, sex, and undergoing diagnostic tests. Conclusions: A high incidence of subepidermal gaps or blisters was seen in middle-aged individuals and females. The compliance rate of the initial clinical diagnosis with the final diagnosis based on pathological reports was high. Our findings emphasize the importance of histopathological examination and the complementary role of direct immunofluorescence and salt split in diagnosis.

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