Neuroectodermal tumours of the sino-nasal tract: a clinicopathological study of 18 cases with a newly proposed revised classification and a brief review of literature.

AIM: Sino-nasal tract tumours constitute 3% of the head and neck malignancies. Among these tumours, neuroectodermal tumours are rare with histo-morphological and immunohistochemical overlap making them a challenge for the pathologist. We included Ewing's/PNET, olfactory neuroblastoma (OFN), mucosal malignant melanomas (MMM), Melanotic neuroectodermal tumour of infancy (MNTI), small-cell neuroendocrine carcinoma (SNEC), and the newest entity Adamantinoma like Ewing's sarcoma (ALES) as part of the neuroectodermally derived tumours of the sino-nasal tract. The last three entities were added to the existing ones, which also has been emphasized in this paper. METHODS AND RESULTS: A comprehensive analysis was done on all neuroectodermally derived tumours from 2016 to 2020. A total of 18 cases were collected, which included OFN (10 cases), SNEC (2 cases), MMM (2 cases), Ewing's/PNET (2 cases), MNTI (1 case), and ALES (1 case). The most common presentation in NE tumours was nasal obstruction (80-100%). Except for OFN, all other tumours were confined to the nasal and paranasal sinuses. 4/10 cases of OFN showed orbital extension. Cervical lymph-node metastasis was seen in 50% of cases of SNEC and MMM groups. An array of relevant immune-histochemical markers were performed. The marker expression was very subtle among the groups. On follow-up, recurrence was seen in the OFN and MMM groups in 30 and 50%, respectively. Metastasis was seen in SNEC group (100%) and OFN group (10%). CONCLUSION: As sino-nasal neuroectodermal tumours pose a diagnostic challenge and have different therapies and are prognostically different, the pathologist must be aware of the subtle morphological, immunohistochemical clues which have been dealt with in-depth in this study.

Clinicopathological Profile of Primary Hyperparathyroidism with Special Reference to Ki-67 Labelling Index.

Context: Primary hyperparathyroidism (PHPT) can occur due to a neoplastic process or hyperplasia. While the disease presentation is predominantly asymptomatic in developed countries, this is not the case yet in India. Differentiation of the type of lesion can only be done based on histomorphology but has its own challenges. Immunohistochemical markers like Ki-67 have been studied to aid in diagnosis but data on this is sparse from India. Aims: The aim of this study is to assess the clinical, biochemical and pathological profile of PHPT and to analyse the differences in immunohistochemical marker Ki-67 among the various lesions. Setting and Design: A descriptive study was carried out on 38 PHPT patients who were treated at our institute from January 2011 to March 2021. Materials and Methods: Post-surgery, the causative lesions were categorised as adenoma (31), hyperplasia (5) and carcinoma (2). Clinical, biochemical, radiological and histopathological features of all lesions were collected and analysed. Ki-67 proliferation index was calculated. The various parameters were compared across the three groups of lesions and correlated with Ki-67 index. Results: Out of 38 patients, 37 were symptomatic with skeletal symptoms being the most common followed by renal symptoms. There was no difference in clinical or biochemical parameters among the three types of lesions. Significant negative correlation was seen between serum iPTH and serum 25-OH Vitamin D levels (P0.006) The median Ki-67 index was found to be 0.40% in hyperplasia, 0.49% in adenoma and 5.84% in carcinoma. Conclusion: PHPT still presents as an overtly symptomatic disease in India. Diagnosis of the nature of lesion depends on the accurate application of morphological criteria. A high Ki-67 index was not found to be an absolute marker of carcinoma, as it was also seen in a small proportion of atypical adenomas.

Diagnostic utility of immunohistochemical markers alpha methyl acyl coA racemase (AMACR) and Ets related gene (ERG) in prostate cancer.

OBJECTIVES: To study the sensitivity and specificity of IHC markers AMACR and ERG in prostatic adenocarcinoma. METHODS: The study was a prospective one and samples were collected from August 2014 to June 2016. A total of 186 samples were obtained from the Department of Urology, in which 112 of these were benign prostatic hyperplasia (BPH), and 71 were prostatic adenocarcinoma. The adenocarcinoma cases were evaluated by two histopathologists, and appropriate Gleason score was given according to the modified ISUP Gleason grading system (2016). IHC markers AMACR & ERG were performed on the adenocarcinoma cases and their sensitivity and specificity were calculated. RESULTS: AMACR was a highly sensitive and specific marker for detecting prostatic carcinoma with a sensitivity and specificity of 95.8% and 96.5% respectively. ERG was a very specific marker with poor sensitivity in detecting prostate cancer. The sensitivity and specificity of ERG were 35.2% and 100% respectively. ERG expression decreased with increasing Gleason grade, PSA level, and tumour volume, which was statistically significant while the association of AMACR with Gleason grade or with tumor volume was not significant. CONCLUSION: ERG is a marker of early prostatic carcinogenesis and tumors may be positive or negative subtypes. Special histomorphologic features like perineural invasion, glomerulations, and intraluminal blue mucin were also studied. AMACR was a highly sensitive marker for detecting prostatic adenocarcinoma, while ERG was highly specific.

Malakoplakia of Endometrium with Osseous Metaplasia on Evaluation of Postmenopausal Leukorrhea: A Rare Case Report.

Malakoplakia is a chronic xanthogranulomatous condition that affects the genitourinary tract reported earlier as urinary granulomas and pelvic masses. We report a different clinical manifestation of malakoplakia presenting as postmenopausal pyometra. A 64-year-old postmenopausal female presented with foul-smelling vaginal discharge with a past history of induced abortion, followed by dilatation and evacuation. On examination, abdomen was soft, vaginal examination revealed pus discharge, parous size uterus with free fornices, and pap smear ruled out malignancy. Ultrasonography revealed linear, echogenic structures in the endometrial cavity suspicious of bony spicules with fluid around. Hysteroscopy revealed congested endometrium with multiple pieces of shredded bone-like structures that were removed followed by curettage. Histopathological examination was suggestive of malakoplakia with osseous metaplasia. Retained bony spicules can cause chronic granulomatous inflammation that may become symptomatic postmenopause due to absent cyclical shedding. This is the first reported case of malakoplakia of uterus following retained bony spicules.

Cholemic Nephrosis: An Autopsy Study of a Forgotten Entity.

OBJECTIVE: The aim of the study is to do a clinicopathologic study of post mortem kidney biopsies with significant deposition of bilirubin pigment within tubular epithelial cells and in the lumen of distal tubules as a bile cast. MATERIAL AND METHOD: All post mortem specimens with acute tubular necrosis, with the presence of bile casts in tubules or bile pigment deposition in the tubular epithelium during the period 2015-2018 were examined for gross and histopathology along with biochemical parameters and viral markers. RESULTS: Bile casts with sloughed renal tubular epithelial cells and occasional macrophages were present in the distal convoluted tubule in 78.6% of biopsies (11/14). The plugging of distal convoluted tubule with casts was similar to that seen in myeloma and myoglobin cast nephropathies. Bilirubin pigment deposition was present in 35.7% (5/14) of cases. The frequency of bile casts in each biopsy was variable and it did not have any association with serum bilirubin levels or etiology of liver dysfunction. A striking difference from earlier studies is the high number of toxin-induced liver damage including six cases of paraquat and 2 cases of yellow phosphorus poisoning. CONCLUSION: This study proves importance of the bile cast nephropathy as a reason for kidney injury, especially with varied hepatotoxic etiologies, especially paraquat and yellow phosphorus.

Recurrent leiomyoma of the vulva mimicking peripheral nerve sheath tumour.

Leiomyoma, a benign monoclonal tumour, is very rarely found in extrauterine sites, especially in the vulval region. Histopathology of the soft tissue tumours affecting this region is similar and immunohistochemistry (IHC) may be essential to confirm the diagnosis. We report a case of a 63-year-old postmenopausal woman who presented with a recurrent vulval mass involving the clitoris and left labia majora with suspected urethral involvement. Although the wedge biopsy was reported as peripheral nerve sheath tumour, staining with smooth muscle actin clinched the diagnosis of leiomyoma, highlighting the importance of IHC for diagnosis.

Sertoliform Endometrioid Carcinoma of Ovary Presenting as Abdominal Wall Abscess.

Sertoliform endometrioid carcinoma (SEC) is a rare epithelial ovarian cancer which may be mistaken for sex cord tumour on microscopy. Immunohistochemistry and extensive sampling of tumour to look for areas of conventional endometrioid carcinoma are important to confirm the diagnosis of SEC. We report a case of SEC of ovary in a postmenopausal lady who presented to us with abdominal wall abscess in epigastric region.

Spontaneous tumour lysis syndrome associated with non-Hodgkin's lymphoma--a case report.

Spontaneous tumour lysis syndrome is characterized by hyperuricemia, hyperkalemia, hyperphosphatemia, metabolic acidosis and hypocalcemia, that occur even prior to the treatment of a neoplasm. This rare occurrence was encountered in a patient with non-Hodgkin's lymphoma (NHL), of follicular cell type. Conservative but intensive treatment led to complete resolution. Subsequent chemotherapy was well tolerated.

Cytologic aspects of an interesting case of medullary thyroid carcinoma coexisting with Hashimoto's thyroiditis.

Among primary thyroid neoplasms, papillary thyroid carcinoma (PTC) and primary thyroid lymphoma (PTL) are known to coexist and are pathogenetically linked with Hashimoto's thyroiditis (HT). However, HT occurring in association with medullary thyroid carcinoma (MTC) is rarely documented. We report here an interesting case. A 34-year-old female with a solitary thyroid nodule underwent fine needle aspiration cytology (FNAC) that was interpreted as "MTC with admixed reactive lymphoid cells, derived possibly from a pretracheal lymph node." Total thyroidectomy specimen showed "MTC with coexisting HT." At a later stage, a follow-up FNAC from the recurrent thyroid swelling showed features consistent with HT. As an academic exercise, the initial smears on which a diagnosis of MTC was offered were reviewed to look for evidence of coexisting HT that showed scanty and patchy aggregates of reactive lymphoid cells without Hürthle cells. Our case highlights an unusual instance of MTC in concurrence with HT that can create a tricky situation for cytopathologists.

A study of fibroepithelial tumours of the breast.

Cytological parameters were studied in 18 fibroepithelial tumours of the breast including 8 benign phyllodes, 1 malignant phyllodes, and 9 fibroadenomas between 1999 to 2001. Cytology smears were studied without knowledge of previous cytodiagnosis by 3 different observers. Histopathologic parameters were studied in 18 fibroepithelial tumours by 3 observers. Cyto-histological correlation was carried out. Cytological criteria by Chinoy et al (1999) were utilized to give revised cytodiagnosis. Initial cytodiagnosis and revised cytodiagnosis were correlated with histopathological diagnosis. 8/9 cases of fibroadenoma (88%) studied were correctly diagnosed on FNAC after semiquantitative team approach as opposite to 44% initial cytodiagnosis. Cytological distinction between benign phyllodes and fibroadenoma was improved after semiquantitative approach to various parameters. Diagnosis of malignant phyllodes posed no problems in cytodiagnosis. Present study supports the role of FNAC in distinguishing benign from malignant phyllodes and distinguishing fibroadenoma from phyllodes tumour. The latter being a rare fibroepithelial tumour, its study is interesting.

Plasma cell leukemia--a case report.

Plasma cell leukemia, occurring either de novo or in patients with long standing multiple myeloma, is the least common type of plasma cell dyscrasia. Histogenetically plasma cell leukemia is derived from terminally differentiated B cells. It is diagnosed by presence of absolute plasma cell count >2000/cm or >20% circulating plasma cells. Two cases of plasma-cell leukemia are reported here. Clinical spectrum and course of the disease, are discussed. Both cases (of primary plasma cell leukemia) had abrupt onset of disease, poor response to therapy and short survival time. Both presented with fatiguability and weakness. Evidence of organomegaly and radiological and hematological evidence of plasma cell leukemia, with thrombocytopenia and evidence of visceral involvement with leukemic cells, was seen in one case.

Malignant transformation in a mature teratoma with metastatic deposits in the omentum: a case report.

Malignant transformation of a mature cystic teratoma (MCT) is a very rare complication with an incidence of 0.17-2%;. The most common form of malignant transformation of the MCT is squamous cell carcinoma. Other tumors arising in MCT include basal cell carcinoma, sebaceous tumor, malignant melanoma, adenocarcinoma, sarcoma, and neuroectodermal tumor. However malignant transformation with metastatic deposits in the omentum is extremely rare. The present case highlights the rarity of the occurrence of an omental deposits in a case of mature cystic teratoma with malignant transformation.

Clinico-aetiologic profile of macrocytic anemias with special reference to megaloblastic anemia.

PURPOSE OF STUDY: This study was conducted to study the clinical and laboratory parameters in patients with macrocytic anemia and to determine the etiology of macrocytic anemia with special reference to megaloblastic anemia. MATERIALS AND METHODS: This study was a cross-sectional descriptive study carried over a period of 18 months on 60 adult patients (age ≥13 years) of macrocytic anemia. Macrocytic anemia was identified when peripheral blood examination showed anemia with a mean red blood corpuscular volume of >95 fl. RESULT: The most common cause of macrocytic anemia was megaloblastic anemia (38.4%). The major causes of nonmegaloblastic macrocytic anemia were primary bone marrow disorders (35%), liver diseases (15%) and hemolytic anemia (8.3%). There was a significant male preponderance in the study (65%). The megaloblastic anemias observed were due to either vitamin B(12) deficiency (78.3%) or combined B(12) and folate deficiency (21.7%). A significant proportion of non-vegetarians (73.9%) had megaloblastic anemia. Patients with an MCV of >110fl were more likely to have megaloblastic anemia (p value 0.0007). Three patients (mean age 55 years) with a megaloblastic marrow did not respond to vitamin replacement and were found to have myelodysplastic syndrome. CONCLUSION: Megaloblastic anemia due to Vitamin B(12) or folate deficiency remains the most important cause of macrocytic anemia. In settings with limited laboratory facilities, a therapeutic trial of vitamins B(12) or folic acid is useful in determining the specific vitamin deficiency.