Clinicopathological study of adrenal pheochromocytoma and extra-adrenal paragangliomas with reference to GAPP and PASS scoring systems.

BACKGROUND: The presence of distant metastasis is known to drastically reduce survival of adrenal pheochromocytoma (PH) and extra-adrenal paraganglioma (PGL). Therefore, predicting malignant potential has an immense impact on prognosis. Pheochtomocytoma of adrenal gland scaled score (PASS) and the grading of adrenal pheochromocytoma and paraganglioma (GAPP) score are two histological algorithms used to predict metastatic potential, but neither has been regarded as 'gold-standard'. Both these scoring systems are yet to be validated. Here, we tried to validate the association of GAPP/PASS scores with disease outcome and strength of association between individual GAPP/PASS parameters with prognosis. MATERIALS AND METHODS: This was a prospective study comprising 22 pheochromocytomas and eight paragangliomas. GAPP score was calculated in paraganglioma cases, and both GAPP/PASS scores were calculated for pheochromocytomas. Disease outcome was then tallied with risk stratification of the GAPP/PASS scoring system. Succinate dehydrogenase B (SDHB) immunohistochemistry was done in 15 cases to see its impact on prognosis. RESULTS: The common PASS parameters associated with malignancy were 'high cellularity', 'tumor cell spindling' and 'extension into adipose tissue'. PASS score showed high sensitivity and negative predictive value but low specificity and positive predictive value. Similarly, GAPP score also showed high sensitivity and negative predictive value but low specificity and positive predictive value. CONCLUSION: In our study, GAPP/PASS scores successfully segregated tumor with low malignant potential from tumor with higher risk of metastasis, although specificity of GAPP was more than PASS. We also found that addition of objective parameters like SDHB immunohistochemistry may further increase the specificity of the existing scoring system.

Sustainable and Affordable Synthesis of (Deuterated) N-Methyl/Ethyl Amines from Nitroarenes.

The sustainable and affordable synthesis of (deuterated) N-methyl/ethyl amines directly from nitroarenes is disclosed. The reaction is catalyzed by a single-site manganese catalyst and involves tandem dehydrogenation, transfer hydrogenation, and borrowing hydrogenation. This catalytic protocol is highly selective and provides a facile method for the large-scale synthesis of a series of N-CH3/N-CD3 aniline derivatives with excellent functional group tolerance.

Diagnostic and prognostic utility of SF-1 in adrenal cortical tumours.

Background and Aims: Superior imaging techniques have increased the recognition of adrenal pathology. Distinguishing benign from malignant adrenocortical tumors is not always easy. Several criteria and immunohistochemical markers have been discovered which help to differentiate between adrenocortical adenoma (ACA) and adrenocortical carcinoma (ACC). Our aim here was to evaluate the diagnostic and prognostic role of steroidogenic factor-1 (SF-1) in adult adrenocortical tumors (ACT) diagnosed using the Weiss criteria. In this cohort, we have also analyzed Ki67 and p53 expression and the extent of agreement between SF-1 and Ki-67. Methodology: This was a retrospective, observational study comprising 24 cases of adult ACT over 10 years. Immunohistochemical staining for SF-1, Ki67, and p53 was done in all the cases, and the results correlated with the morphological diagnosis made using Weiss criteria. Results: SF-1 was 100% sensitive and 80% specific as a marker of malignancy. Increased SF-1 expression correlated with worse survival. There was a moderate degree of agreement between Ki-67 labeling-index and SF-1 as a marker of malignancy with the kappa coefficient being 0.75. The sensitivity of p53 was lower than Ki67 in diagnosing ACC. Conclusion: In adult ACTs, SF-1 has diagnostic significance and prognostic implication. SF-1 is a crucial, dosage-dependent survival factor in ACC. There is a moderate extent of agreement between Ki-67 and SF-1 as a marker of malignancy.

Thanatophoric dysplasia type 1 with temporal lobe dysplasia: Report of a case along with differential diagnosis.

Thanatophoric dysplasia type 1 (TD1) is a lethal form of osteochondral dysplasia due to mutation of FGFR3 gene. In addition to severe shortening of the limbs there is temporo-occipital lobe dysplasia along with a range of other CNS anomalies. In this report we describe the radiological and anatomical features at autopsy in neonate with TD1 along with the CNS anomalies. We have also summarized the key distinguishing features of TD1 from other common types of osteochondral dysplasia. An accurate diagnosis is important for genetic counseling and impact on future pregnancies.

Double parasite in appendicectomy specimen: Causal factor or incidental finding?

Appendicitis is the most common cause of acute abdomen leading to invasive surgery. While the mainstay treatment of appendicitis is appendicectomy, there are a few conditions where conservative medical treatment cures the patient. One such condition is intestinal parasitosis, where only pharmacotherapy causes remission. Although Enterobius vermicularis is the commonest parasite in this regard, others such as Schistosoma, Taenia, Ascaris and Trichuris can also be found. Coexistence of two parasites in appendicectomy specimen is distinctly rare. We present one such case here of double infestation with Enterobius and Trichuris in the appendix.

Composite phaeochromocytoma with malignant peripheral nerve sheath tumour: A case report with summary of prior published cases.

Composite phaeochromocytomas (CP) are extremely uncommon adrenal medullary tumours where phaeochromocytoma coexists with another adrenal medullary tumour also of neural crest origin. CP includes combination of phaeochromocytoma along with a component of neuroblastoma, ganglioneuroblastoma, ganglioneuroma, benign nerve sheath tumour or a malignant peripheral nerve sheath tumour (MPNST). Here we describe the morphological and immunohistochemical details of a case of CP with MPNST in a 30 years old lady, without history of neurofibromatosis. Only 6 cases of CP with MPNST have been reported so far. We have tabulated a summary of these prior published cases of phaeochromocytoma with MPNST. To our knowledge, this is the first literature review describing the clinico-pathological characteristics of these rare tumours.

Manganese(I)-Catalyzed Sustainable Synthesis of Quinoxaline and Quinazoline Derivatives with the Liberation of Dihydrogen.

Direct synthesis of N-heterocycles via the acceptorless dehydrogenative coupling is very challenging and scarcely reported under 3d transition-metal catalysis. Here, we have developed an efficient Mn(I)-catalyzed sustainable synthesis of various quinoxalines from 1,2-diaminobenzenes and 1,2-diols via the acceptorless dehydrogenative coupling reaction. Further, this strategy was successfully applied for the unprecedented synthesis of quinazolines by the reaction of 2-aminobenzyl alcohol with primary amides. The present protocol provides an atom-economical and sustainable route for the synthesis of various quinoxaline and quinazoline derivatives by employing an earth-abundant manganese salt and simple phosphine-free NNN-tridentate ligand.

Diagnostic utility of cytology smears and cell block in adrenal lesions.

BACKGROUND: Cytology of the adrenal gland is a less performed technique even in tertiary care centres; yet cytological evaluation is an important diagnostic tool for assessment of adrenal lesions. Our objective was to evaluate the diagnostic utility of FNAC smears and cellblock with immunohistochemistry (IHC) in lesions of the adrenal. MATERIAL AND METHODS: We had a total of 50 cases over a period of 2 years where both FNAC smears and cellblocks were taken. The smears and cellblocks were examined for adequacy. They were subsequently categorised into four groups: unsatisfactory, benign, suspicious of malignancy and malignant. The diagnostic accuracy of FNAC smears and cellblock with IHC were evaluated and compared, taking histopathology, wherever available, as the gold standard, RESULT: We had 50 cases with age ranging from 6 to 53 years with a median of 7.5 years. Of these, 54% were cytologically malignant and neuroblastoma was the commonest lesion. Histopathology was available in 23 cases only, where the diagnostic accuracy was evaluated. The sensitivity and specificity of FNAC smear was 100% and 85.71%, respectively whereas the sensitivity and specificity of cellblock with IHC was 100% and 92.86%, respectively. CONCLUSION: Cellblock together with IHC provides a higher degree of specificity, reduces the unsatisfactory rate and improves the diagnostic accuracy in lesions of the adrenal gland. Immunohistochemistry is an important adjunctive tool.

Nickel-Catalyzed Chemoselective Acetalization of Aldehydes With Alcohols under Neutral Conditions.

A molecularly defined NiII -complex catalyzing the chemoselective acetalization of aldehydes with alcohols under neutral conditions is reported. The reaction is general, efficient and showed a wide substrate scope (including aliphatic aldehydes) as well as excellent functional group tolerance. Reusability of the present nickel catalyst is also demonstrated.

Manganese catalyzed N-alkylation of anilines with alcohols: ligand enabled selectivity.

Ligand enabled Earth-abundant manganese catalyzed N-alkylation of amines with alcohols via a hydrogen auto-transfer strategy is reported. The choice of the ligand plays a significant role in the alcohol reactivity (aliphatic or aromatic) toward N-alkylation reactions.

Manganese-Catalyzed Direct Conversion of Ester to Amide with Liberation of H2.

A simple and efficient Mn-catalyzed acylation of amines is achieved using both acyl and alkoxy functions of unactivated esters with the liberation of molecular hydrogen as a sole byproduct. The present protocol provides an atom-economical and sustainable route for the synthesis of amides from esters by employing an earth-abundant manganese salt and inexpensive phosphine-free tridentate ligand.