The teaching pathologists association of Mumbai: A legacy that lives on.

ABSTRACT: The Teaching Pathologists Association of Mumbai was started by two doyens of pathology, Dr V. R. Khanolkar and Dr. P. V. Gharpure. They were the heads of the departments of pathology at Seth G S Medical College and Grant Government Medical college. The first meeting of the association was held in July 1935 at the Grant Medical College. The idea of the meetings was to know about the work done in other institutes and was attended by staff and students of these institutes. Soon Haffkine Institute joined, followed by Tata Memorial Hospital, TN Medical College, and Lokmanya Tilak Medical College in the late 1960s, with the latest entrant being HBT Medical College in 2023. The meetings that started in 1935 have continued till date (2023) with over 500 meetings being conducted so far. The nature of the meetings was in the form of a presentation by a postgraduate or faculty member. It was left to the discretion of the head of the department. This has been a unique association with no formal office bearers and runs purely on the understanding and cooperation of the senior teachers of these institutes. It has nurtured the spirit of mutual cooperation among the various medical institutes in Mumbai with the exchange and sharing of views and material. This article describes the beginning and subsequent growth of this association, the likes of which are yet to be seen elsewhere.

Choroidal ganglioneuromas in Francois variant neurofibromatosis-1: A rare retinoblastoma mimic.

Neurofibromtosis-1 (NF-1) is the commonest oculo-neuro-cutaneous syndrome with multiple ocular manifestations. Reporting three children who presented with unilateral glaucoma (buphthalmos), ipsilateral facial hemihypertrophy, and eyelid plexiform neurofibroma: completing the triad of François syndrome, a rare NF1 variant. Two presented with leukocoria and were referred to as retinoblastoma suspects. Histopathology showed ganglioneuroma, a benign choroidal tumor, associated with NF-1, which does not need treatment. Knowledge of this rare condition avoids misdiagnosis of retinoblastoma, prevents aggressive management, and the associated psychological impact.

Epidemiology of Pediatric Renal Diseases and its Histopathological Spectrum - A Single-Center Experience from India.

Pediatric renal biopsy is an uncommon event, and the spectrum of the disease is evaluated and managed mostly on the clinical grounds. Compared to adults, the indications for renal biopsy in pediatric population are very few. We reviewed the pediatric renal biopsies received at our tertiary center in Mumbai, India, over a period of six years to study the incidence of various medical renal diseases, their spectrum on histology and its correlation with electron microscopy (EM). A total of 65 pediatric renal biopsies in the age group of 0-12 years were evaluated over a period of six years. The mean age of our patients was 7.9 years, with a median of 8.8 years with a male-to-female ratio of 1.3:1. The overall most common indication for biopsy was nephrotic syndrome (NS) including steroid-resistant NS, followed by proteinuria and nephritic syndrome. Majority of the lesions included in the study were primary glomerular disease (71%) while secondary glomerular disease amounted to 18%. The spectrum of disease includes minimal-change disease (MCD) (27.7%), followed by membranoproliferative glomerulonephritis (MPGN) (15.38%), focal segmental glomerulosclerosis (FSGS) (9.23%), lupus nephritis (7.7%), hemolytic uremic syndrome (7.7%), MPGN (6.15%), advanced renal disease (6.15%), membranous glomerulonephritis (3.07%), and crescentic glomerulonephritis (3.07%). This study is an important contribution to the epidemiology of pediatric renal disease spectrum in the Indian population. We conclude that MCD is the most common pathology seen in pediatric age group, with NS as the most common indication for biopsy. There is a steady increase in the incidence of FSGS in the pediatric population with frequent relapses and an increase in the incidence of steroid resistance. However, with the use of immunofluorescence and EM, an accurate diagnosis is possible, so an early renal biopsy should be planned in nonresponding cases and at times even before starting the treatment for appropriate treatment.

Alobar Holoprosencephaly Associated with Meningomyelocoele and Omphalocoele: An Unusual Coexistence.

Holoprosencephaly is a rare congenital disorder which results from failure of cleavage or incomplete differentiation of the forebrain structures at various levels or to various degrees. Depending on the degree of involvement, it is classified into 4 types: Alobar, Semilobar, Lobar and Middle interhemispheric fusion variant. A male child was born to 28-year-old female at 34 weeks of gestation. The mother on antenatal follow-up was detected to have a fetus with multiple congenital anomalies on Ultrasonography (USG) done at 34weeks of gestation. The baby died after 12 hours of birth. A complete autopsy was performed. On external examination, multiple congenital anomalies were seen including cleft lip and palate, absent nasal bridge, proptosis of right eye, micropenis, left undescended testis, bilateral rocker bottom feet, omphalocele and sacral meningomyelocele. Internal examination of the brain revealed hydrocephalus and features of alobar holoprosencephaly. This case is presented for its rarity. In addition, it is unusual for a fetus with alobar holoprosencephaly to survive till term as this is the most severe type. Though facial malformations are usually present in a case of holoprosencephaly, its association with sacral meningomyelocele and omphalocele has rarely been described in literature.

A Survivor of Neonatal Intestinal Mucormycosis.

Gastrointestinal mucormycosis (GIM) is a rare opportunistic fungal infection. One third of all patients are children and of these, 50% are infants. The most common clinical mimic is necrotizing enterocolitis (NEC). It has to be differentiated from this entity as the treatment is entirely different. High index of suspicion by the clinicians and the pathologists aids in early diagnosis and immediate treatment. If untreated, it has a frequent fatal outcome. Very few survivors of GIM are found in literature. We report a rare case of a surviving neonate of GIM.

An Unusual Presacral Cyst in An Infant.

The neurenteric cyst is a rare developmental lesion arising as a result of persistence of the neurenteric canal. It usually presents in the second and third decade. Lesions occurring in children are rare. Majority of the cases presented with signs of spinal cord involvement. This is a rare case of neurenteric cyst in an infant who presented with a presacral mass. A nine-month-old female had an antenatally diagnosed sacrococcygeal lesion. Radiology suggested a diagnosis of sacrococcygeal teratoma. Histology was typical of a neurenteric cyst. We report this case to create awareness about this rare cystic lesion and inclusion of this entity in the differential diagnosis of cystic masses in children at this location.

OVOTESTICULAR DISORDER OF SEX DEVELOPMENT: A SINGLE-CENTER EXPERIENCE.

OBJECTIVE: Ovotesticular disorder of sex development (OT DSD) is a rare disorder of sex development characterized by the presence in the same individual of both histologically proven testis and ovary. There are scant data from the Indian subcontinent regarding this disorder. The aim of this study was to describe the clinical, biochemical, imaging, cytogenetic, surgical, and histopathologic findings and outcomes of patients with OT DSD from Western India. METHODS: The records of patients referred to our center for disorders of sex development between 2005 and 2013 were reviewed, and 7 patients were found to have histologically proven OT DSD. RESULTS: The median age at presentation was 8 years (range, 2 months to 25 years). Clinical presentation varied from genital ambiguity and inguinal swelling at birth to gynecomastia and cyclical hematuria after puberty. Karyotype was 46, XX in 6 patients and 46, XY in 1 patient. All patients underwent pelvic ultrasonography, laparoscopy, and surgery for removal of gonads not congruous with the chosen sex of rearing. Gender assignment for all the patients was done by the parents at birth, which was mainly influenced by the external genitalia and sociocultural influences, with 5 out of the 7 patients being reared as males. There was no evidence of gonadal tumors in our study. CONCLUSION: OT DSD should be considered as one of the differential diagnoses in cases of ambiguous genitalia with nonpalpable or asymmetrical gonads, pubertal gynecomastia, and cyclical hematuria, irrespective of the karyotype or internal genitalia.

Fetus in fetu: an institutional experience.

The objective of this article is to present an analysis of cases of fetus in fetu (FIF) diagnosed at our institute and to compare our data to that of the published literature. This is a retrospective analysis of cases of FIF diagnosed at our institute from January 2000 to December 2012. Details of clinical and investigational data pertaining to cases of FIF were retrieved from hospital records, and were analyzed and summarized. Literature was reviewed and our data were compared to that of published cases. We evaluated seven cases (4 males and 3 females) of FIF during the study period. The age of patients ranged from two days to 15 years. The most common site of occurrence in our study was the retroperitoneum, with the most common presenting feature being an abdominal mass. Radiology showed a solid-cystic mass. Pathologic findings were diagnostic of FIF. Most findings of our study matched with those reported in literature. The condition of FIF must be included in the differential diagnosis of abdominal masses, especially in children. This entity must be differentiated from the more commonly occurring teratoma, which can be done based on its unique clinical, radiologic, and pathologic characteristics. To our knowledge, this is the longest series of cases of FIF reported from a single institute.

Sirenomelia with oesophageal atresia: a rare association.

We are reporting a rare case of sirenomelia with oesophageal atresia. Sirenomelia is a lethal sporadic defect of which lower gastrointestinal tract anomalies are characteristic findings. Respiratory and upper gastrointestinal tract malformations like oesophageal atresia occur in about 20-35% of cases. Though its occurrence has been described, it has been reported only rarely. This report aims at describing this uncommon association along with its histological features.

To observe the intensity of the inflammatory reaction caused by neonatal urine and meconium on the intestinal wall of rats in order to understand etiology of intestinal damage in gastroschisis.

OBJECTIVES: The aim of this experimental study was to observe the intensity of the inflammatory reaction caused by neonatal urine and meconium on the intestinal wall of rats to better understand etiology of intestinal damage in gastroschisis. MATERIALS AND METHODS: A total of 24 adult Wistar rats were used as experimental models to simulate the effect of exposed bowel in cases of gastroschisis. The peritoneal cavity of the rats was injected with substances which constitute human amniotic fluid to study the effect on the bowel. Sterile urine and meconium were obtained from newborn humans. The rats were divided into four groups according to the material to be injected. In Group I (Control group) 3 mL of distilled water was injected, in Group II (Urine group) 3 mL of neonatal urine was injected, in Group III (Meconium group) 5% meconium suspension was injected, while in Group IV, a combination of 5% meconium suspension and urine was injected. A total of 3mL solution was injected into the right inferior quadrant twice a day for 5 days. The animals were sacrificed on the 6(th) day by a high dose of thiopentone sodium. A segment of small bowel specimen was excised, fixed in paraffin, and stained with hematoxylin-eosin for microscopic analysis for determination of the degree of inflammatory reaction in the intestinal wall. All pathology specimens were studied by the same pathologist. RESULTS: The maximum bowel damage was seen in Group II (Urine group) in the form of serositis, severe enteritis, parietal necrosis, and peeling. A lesser degree of damage was observed in Group III (Meconium group) as mild enteritis (mild lymphoid hyperplasia). The least damage was seen in Group IV (Combination of meconium and urine) and Group I (Control group). CONCLUSION: The intraabdominal injection of neonatal human urine produces significant inflammatory reactions in the intestinal wall of rats.

Cyanotic nephropathy--a morphometric analysis.

OBJECTIVE: Nephropathy is a known complication in cyanotic congenital heart disease (CCHD). This study was undertaken for an objective analysis of histopathological changes of cyanotic nephropathy at autopsy. DESIGN: Retrospective case records studied. SETTING: Tertiary care teaching hospital affiliated to medical college in Mumbai, India. PATIENTS AND METHODS: The renal histopathological findings of 50 consecutive autopsies in patients with CCHD were compared with 25 age-matched controls. The Bowman's capsular, glomerular tuft, and hilar arteriolar diameters were measured morphometrically. Statistical analysis was performed using unpaired t-test. A P value equal to or less than .05 was considered significant. RESULTS: Among the 50 autopsied cases of CCHD, there were 35 males and 15 females, with a mean age of 4.64 years. The renal changes observed were glomerulomegaly, glomerulosclerosis, periglomerular fibrosis, hyperplastic arteriolosclerosis, and interstitial fibrosis. The objectively measured parameters were higher in cases as compared with controls in all age groups, and further these were also found to be higher in patients having decreased pulmonary arterial blood flow than those having normal to increased pulmonary arterial blood flow. The difference in Bowman's capsular and glomerular tuft diameters was statistically significant in the neonates and children in the age groups, 1-5 years and above 10 years. The difference in hilar arteriolar diameter was statistically significant for all age groups except neonates. CONCLUSION: Patients with CCHD show significant changes in the kidneys as assessed morphometrically, leading to renal dysfunction, and the age of the patients plays a role in their development.

Bilateral Epstein-Barr virus-associated adrenal leiomyomas in a child without an established immunodeficiency.

Adrenal leiomyomas are rare, bilateral ones being rarer. Literature available on these rare tumors documents only 4 cases in children less than 12 years of age. Each case has been associated with acquired immune deficiency syndrome or some other immunodeficiency state. Here we present a rare case of large, bilateral, adrenal leiomyomas in a child with no known immunodeficiency. An 11-year-old girl with a past history of herpes zoster (1 year before the present complaints) was admitted with abdominal pain of 2 months' duration. Radiology revealed bilateral adrenal neoplasms, probably bilateral pheochromocytoma. Histology showed bilateral adrenal leiomyomas that were Epstein-Barr virus associated. We report this case to draw attention to the occurrence of a common pathologic entity at an uncommon site in a setting of no definite known immunodeficiency.

Multifocal nephrogenic adenoma ­ a mimicker of malignancy.

The authors report a multifocal nephrogenic adenoma of urinary bladder in an 11-y-old girl. Nephrogenic adenomas occur almost exclusively in the urinary bladder in children and are rarely multifocal. Less than 30 cases of nephrogenic adenomas have been reported in children and very few of these are multifocal. This report aims at drawing attention to this rare entity in children.

Key cytological findings in FNA from infantile digital fibromatosis.

BACKGROUND: Infantile digital fibromatosis (IDF) or inclusion body fibromatosis is a benign proliferation of fibroblastic and myofibroblastic cells. Its most common site is the digits of young children and it is named for the intracytoplasmic inclusions that are detected in lesional cells. CASE: A two and a half-year-old male child presented with a single flesh-colored nodule on the dorsal aspect of his right little toe since the sixth month of life. FNAC from the lesion showed characteristic cytomorphological features of IDF. CONCLUSION: IDF is a rare lesion occurring in children or infants. There are many case reports describing histopathological features of IDF. To our knowledge, the typical inclusion bodies as cytomorphological findings in IDF have not been described in the literature. This is a rare case diagnosed on cytology and confirmed on histopathology.

Giant adrenal myelolipoma.

Adrenal myelolipoma is a rare benign tumor composed of adipose and hematopoietic tissue. Most tumors are small in size and incidentally detected. We report a case of giant adrenal myelolipoma weighing 2200 gms which was diagnosed on radiology as a liposarcoma. This case is unusual in view of the large size and presence of bony spicules. To the best of our knowledge, not more than 10 giant adrenal myelolipoma cases have been reported in literature.

Primary hyperparathyroidism: a clinicopathological experience.

BACKGROUND: Parathyroid neoplasms form a small percentage of head and neck neoplasms. Primary hyperparathyroidism is caused by parathyroid adenomas, hyperplasia or, rarely, a carcinoma. MATERIALS AND METHODS: This is a retrospective study of 48 parathyroidectomies received in the Department of Pathology of a major teaching hospital over a period of 10 years. Clinical, biochemical and radiological details were retrieved from medical records. Information regarding routine gross and microscopic examination findings (including frozen section and paraffin sections) was retrieved from departmental records. RESULTS: We had 43 adenomas, three hyperplasias and two carcinomas. The most common age group was 21-30 years. The female:male ratio was 2.5:1. Most patients presented with skeletal manifestations. The pre-operative diagnosis was assisted by ultrasonography in 11 cases, computerized tomography of the neck in 10 cases and sestamibi scans in three cases. Intra-operative parathormone monitoring was performed in one case of adenoma. Frozen section was requested in 28 of 48 cases. There was a discrepancy between frozen section diagnosis and paraffin section diagnosis in two cases. CONCLUSION: Histopathologic diagnosis is an important guide to decide the type of surgical management. Although pathologic features of parathyroid carcinoma are diagnostically reliable, those of the more commonly encountered lesions of adenoma and hyperplasia may be overlapping and, therefore, indistinctive, more so if only a single gland is available for examination. Because parathyroid lesions are only occasionally encountered by the surgical pathologist, awareness of the spectrum of histologic features along with knowledge of recent trends in diagnosis and surgical management are important.

The renal pathology in children dying with hepatic cirrhosis.

The aim of this analysis was to observe the pathologic changes in the kidney in 23 children dying of cirrhosis, including five children with renal failure. Besides these pathologic changes, glomerular cellularity and arteriolar wall thickness of these patients were compared with 18 age matched autopsy controls dying of nonhepatic and nonrenal causes. Histologic examination of the kidneys in these cirrhotic children showed significant tubular changes in five patients, diffuse glomerulosclerosis in five, only one child had membranous glomerulopathy or glomerulonephritis and one cirrhotic with serum hepatitis B surface antigen positivity had polyarteritis nodosa. No statistically significant changes were observed in the glomerular cellularity and the arteriolar wall thickness in these cirrhotic children as compared to the controls. However, medial proliferation and hyaline arteriolosclerosis were observed in four cirrhotic children. Tubular bile casts and nephrocalcinosis were the sole lesions causing renal failure in two cases. A combination of glomerulosclerosis, cast formation and arteriolosclerosis was seen in the remaining three patients. Thus the kidney showed a spectrum of pathology in pediatric cirrhosis. The tubular lesions causing renal failure represent acute treatable lesions whereas glomerulosclerosis and arteriosclerosis stand for a more chronic and less reversible renal damage.