Cytomorphology of Warthin-like variant of papillary thyroid carcinoma: A diagnosis not to be missed.

The authors have elaborated the cytological features of Warthin-like variant of papillary thyroid carcinoma (WLPTC) presenting unusually with nodal metastasis in a 43-year-old lady, and which was reported on cytology as papillary thyroid carcinoma (PTC) with lymphocytic thyroiditis.

Successful use of tocilizumab in amyloidosis secondary to systemic juvenile idiopathic arthritis.

Amyloidosis secondary to juvenile idiopathic arthritis is a known complication of poorly controlled systemic juvenile idiopathic arthritis (SJIA), occurring in 1-2% of the patients. The IL-6 inhibitor tocilizumab is effective in controlling systemic signs and symptoms of sJIA and may be of therapeutic benefit in secondary amyloidosis. Herein, we report the clinical timeline of a 10-year boy with sJIA and secondary amyloidosis, who showed a sustained improvement of systemic symptoms and a reduction in proteinuria with tocilizumab. Compared to the data on adult patients affected with the secondary amyloidosis, there are very few reports on therapeutic options for the children affected with SJIA and secondary amyloidosis in the paediatric population. While doing a systematic literature search for writing this review, we could only retrieve nine case reports and one case series of the children affected with SJIA and secondary amyloidosis, including five cases which were treated with tocilizumab. We also looked into the clinical and biochemical response to various agents that have been used in the previous cases, including tocilizumab. The available literature and the present case report suggest that tocilizumab may be considered as a safe and effective option to treat SJIA-related secondary amyloidosis.

Juvenile dermatomyositis with IgA nephropathy: case-based review.

Juvenile dermatomyositis (JDM) is the most common childhood idiopathic inflammatory myopathy (IIM). It is characterized by the classic skin rash in the form of Gottron papules and heliotrope rash, and symmetric proximal muscle weakness. Renal involvement in JDM is rare which includes acute kidney injury and glomerulonephritis. We report a 10-year-old boy with juvenile dermatomyositis and IgA nephropathy. Child responded dramatically to the conventional therapy with steroids and methotrexate for the primary disease, and did not require any additional treatment for his renal disease. Child's primary disease is in remission and has normal urinalysis with normal renal function at 6-month follow-up. We reviewed the literature and found 11 cases of IIMs with renal involvement. Four patients (one JDM, two polymyositis, and one dermatomyositis) had IgA nephropathy out of which three patients responded to the conventional therapy of primary disease and only one patient with polymyositis needed hiking immunosuppression targeted for renal condition. Therapy targeting the underlying disorder is usually sufficient in patients with JDM and secondary IgA nephropathy.

Cytological diagnosis of Langerhans cell histiocytosis: A series of 47 cases.

OBJECTIVE: Langerhans cell histiocytosis (LCH) is a rare disease affecting predominantly children and young adults but can be found in any age group. Diagnosis of LCH is often difficult and can be delayed because of its rarity. The present study highlights the cytomorphological features in a large cohort of cases. An accurate cytological diagnosis may avoid unnecessary biopsy and guide appropriate management. METHOD: Fourty seven (47) cases of LCH diagnosed on cytological material & fine-needle aspiration (FNA) over a period of 14 years (2003-2016) were retrieved from the archives. The cytological smears were evaluated and microscopic findings collected by semi-quantitative assessment done by two different pathologists RESULT: The age at the diagnosis of the patients ranged from 9 months to 28 years. The majority of cases were in the age group of 0-5 years. The most common site was head and neck region, which included cervical lymphadenopathy and scalp swelling. Two cases were diagnosed each from inguinal lymph node and bronchio-alveolar lavage (BAL). Cytological smears in the majority of the cases were moderate to highly cellular (58%) and showing abundant Langerhans cell in (72%) of cases. Areas of necrosis were seen in 38%, while 78% of cases showed giant cells. The majority of cases showed mild eosinophilia (61%), sparse lymphocytosis (83%) and mild neutrophilic infiltration (64%). There were 1-2 mitoses per 10 high power field in 12 cases (25.5%). No abnormal mitoses were identified. CONCLUSION: The presence of cells with features of Langerhans cells associated with the expression of selected immunohistochemical markers allow the diagnosis of LCH on cytological samples, sparing more invasive procedure as a biopsy.

Embryonal Rhabdomyosarcoma of the Prostate: Clinico-Pathological Highlights with Review of Literature.

Rhabdomyosarcoma (RMS) is the third most common extra-cranial sarcoma occurring in childhood, adolescents, and young adults (AYAs); and is rare in adults. Literature about RMS mainly considers RMS in AYAs, either with that in the children or adults, even though histological, molecular, and clinical characteristics of RMS in AYAs are significantly different from either of the two. Herein, we report a case of prostatic embryonal RMS, in a 17-year-old boy, along with the review of literature of prostatic RMS, with emphasis on AYAs. Our patient presented with clinical complaints of acute urinary retention, Grade IV prostatomegaly and, low serum prostate-specific-antigen (0.11ng/dl). The diagnosis was clinched by prostatic biopsy, which revealed diffuse 'small round blue cell' tumour admixed with larger rhabdomyoblasts, displaying positivity for desmin and myogenin, on immunohistochemistry. Clinicians should be mindful that RMS is found in all age groups ranging from childhood to adults; however, the clinical, histological, and molecular features are different. RMS in AYAs is often treated according to the guidelines provided for the paediatric age group. Treatment mostly comprises a multimodality approach, including surgery with/without chemo- and radiotherapy. Prognosis in AYAs is worse than in children but is better than in adults. Thus, early diagnosis gains utmost importance to provide comparatively more probability of rendering treatment and, hopefully, a better quality of life.

Esophageal Melanocytosis.

Esophageal melanocytosis is a rare entity defined by the proliferation of a melanocytic basal layer of the esophageal squamous lining and deposition of melanin in the esophageal mucosa. Esophageal melanocytosis is considered a benign entity of unknown etiology; however, it has been reported as a melanoma precursor. We report a case of esophageal melanocytosis in a diabetic and hypertensive 67-year-old male with recurrent dizziness and syncope for the past 6 months. Given his complaint of dyspepsia, he underwent an upper gastrointestinal endoscopy, in which an esophageal biopsy revealed the diagnosis of esophageal melanocytosis. The definitive diagnosis of esophageal melanocytosis can only be made by histological analysis. The histologic differential diagnoses include melanocytic nevi and malignant melanoma. Therefore, they need to be ruled out.

A Study of Ki-67 Immunostaining in Prostate Carcinomas and Its Correlation with Gleason's Score and Prognosis: An Experience at a Tertiary Centre in the Himalayan Foothills.

Prostate cancer is a significant cause of cancer-related mortality among men worldwide, necessitating the exploration of prognostic biomarkers to aid in accurate risk assessment and treatment decision-making. This cross-sectional study aimed to comprehensively evaluate the role of Ki-67 as a prognostic marker in prostate cancer by examining its association with clinicopathological parameters. A total of 102 archived cases of prostate core biopsy specimens, histopathologically reported as prostate carcinoma, were included in this study. Histopathological grading was conducted using Gleason's scoring and grading system based on morphology. The statistical software "R" was utilized for data analysis. Kruskal-Wallis test and Fisher's exact test were employed to analyze the association between Ki-67 expression and clinicopathological parameters. The study revealed significant correlations between Ki-67 expression and various clinicopathological parameters in prostate cancer cases. High Ki-67 expression levels were associated with higher Gleason scores, increased incidence of perineural invasion, advanced T stages, lymph node metastasis, presence of distant metastasis, and higher prognostic stage groups. The findings of this cross-sectional study support the potential of Ki-67 as a prognostic marker in prostate cancer. The significant associations observed between Ki-67 expression and clinicopathological parameters indicate its usefulness in risk stratification and treatment decision-making. The incorporation of histopathological grading, including Gleason scoring, and analysis of perineural invasion strengthens the validity of the study. Ki-67, in combination with morphological assessments, provides valuable prognostic information for prostate cancer patients.

Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS)/Nesidioblastosis as the underlying cause of recurrent hypoglycemia in a diabetic adult.

Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS), without previous bariatric surgery, is a rare form of hypoglycemia in adult patients and is associated with nesidioblastosis. Adult-onset nesidioblastosis in diabetic patients is rare and histologically identical to "non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS)". Nesidioblastosis is rare in adults and clinically and biochemically mimics Insulinoma. In the literature, there have only been four cases of adult nesidioblastosis that followed diabetes mellitus. We report a case of nesidioblastosis in a 36-year-old diabetic female presenting with dizziness, sweating, and palpitations for three years. Selective non-invasive techniques failed to detect a tumor. Based on the pursuit of an insulinoma, a distal pancreatectomy specimen was received at our laboratory, and a diagnosis of nesidioblastosis was made. She is currently on follow-up with a favorable outcome. The definitive diagnosis of nesidioblastosis is made on a histological basis. The preferred form of treatment is pancreatic surgical resection. Nesidioblastosis should be taken into consideration in cases where diabetes transforms into hyperinsulinemic hypoglycemia.

Hydatid Disease of Tibia Reconstructed with Allograft: A Rare Case Report and Literature Review.

Background: Hydatid disease of bone shows a well-defined, multiloculated lytic lesion with the appearance of a bunch of grapes. The presenting symptoms are pain and swelling with or without pathological fracture. The treatment options include surgery followed by a long duration of albendazole. Removal of the involved bone is required to decrease the chances of recurrences. Case Report: In our study, we have included a case of 28-year-old woman presented with complaints of pain and difficulty in weight bearing over her right lower limb for 2.5 months. Radiograph suggested an eccentric lytic lesion in midshaft of tibia and biopsy revealed granulosus cyst wall, nucleate germinal layer, the brood capsule, and protoscolices with visible hooklets. Patient was subjected to surgery with the excision of cyst along with extended curettage of bone creating a bone defect around the lesion and with anterolateral platting with coverage of bone defect by allogenic bone grafting. Patient was kept on above knee slab with non-weight-bearing mobilization for 6 weeks. Postoperative chemotherapy with Albendazole was given for 3 months. Patient was followed up every 6 weeks for 3 months and every month thereafter on outpatient basis. Return to work and patient satisfaction were excellent. Conclusion: Definitive Surgical management with Preoperative and postoperative chemotherapy seems to be effective to avoid recurrence. The bone defect caused by the disease or surgery can be managed with a bone graft either of autograft or allograft.

Myocardial bridging - Sudden unexpected death of a young girl: A case report.

Myocardial bridging (MB) is a relatively uncommon congenital anomaly where a segment of the coronary artery dips inside the myocardium and takes a tunneled course under a bridge of the myocardium. This leads to the compression of the coronary artery during systole resulting in hemodynamic changes and their clinical manifestations. However, it is an incidental finding but can present with multiple complications like myocardial ischemia, infarction, and sudden death, primarily when associated with other risk factors like left ventricular hypertrophy of the heart. Therefore, a careful examination of the heart is essential for evaluating the clinical significance of the MB. Here, we presented a case of a 30-year-old young female who had a sudden death, and her histological examination of the heart showed MB of left anterior descending coronary artery (LAD).

Primary ovarian leiomyosarcoma in a woman with uterovaginal prolapse.

Primary ovarian leiomyosarcoma is a very uncommon and aggressive neoplasm. We presented a right-sided ovarian leiomyosarcoma in a woman in her late 40s. No case has been described in the literature till now of primary ovarian leiomyosarcoma in a woman with uterovaginal prolapse. A total abdominal hysterectomy with bilateral adnexectomy, metastasectomy, excision of large tumour deposit over small intestine followed by resection with ileo-ileal anastomosis and omentectomy was performed. The diagnosis was made based on morphology along with immunohistochemistry. The patient was given adjuvant chemotherapy during postoperative period. Due to rarity, there is a dearth of information on the clinical behaviour and best treatment options for these tumours. This case report highlighted the importance of clinical awareness and aimed to provide a baseline to guide clinical practice as well as future research.

Growing teratoma syndrome: a surgical conundrum.

Growing teratoma syndrome (GTS) is a tumour growth, which contains mature teratomatous elements during or after chemotherapy for malignant germ cell tumours. Surgery is the only potential treatment option for GTS because these growing teratomas are resistant to chemotherapy and radiation therapy. Extensive surgeries may be needed in GTS with multivisceral resections to achieve no residual disease status. This report presents a case of GTS treated with multiple surgical resections in a woman with malignant immature teratoma ovary in her early thirties; she is disease free after 1 year of treatment.

Rare Floret Like Tyrosine Crystals in Pleomorphic Adenomas of Parotid Gland.

The pleomorphic adenoma arising in the parotid gland is a benign neoplasm that is aptly named because of its histomorphological diversity. The stromal component can contain chondromyxoid material, amyloid, and elastic fibers, along with a few rare reports of crystalline structures present in this tumor. Especially, the crystalline components are rarely encountered or appreciated in routine pathology reporting. Here, we report a case of pleomorphic adenoma of the parotid gland, in which tyrosine-rich crystalloids were identified in abundance and were confirmed with special stains and electron microscopy. Though their exact source is not yet known, crystallization of the stromal or myoepithelial cell secretion has been hypothesized. This comprehensive report is to make the histopathologists aware of this rare morphological observation in pleomorphic adenomas so that more cases are identified and followed-up to reveal the impact of their presence in a subset of pleomorphic adenomas.

Pleomorphic Adenoma with Extensive Squamous and Adipocytic Metaplasia Mimicking as Low Grade Mucoepidermoid Carcinoma on FNAC.

Pleomorphic adenoma (PA) is the most common salivary gland tumor, accounting for 54-76% of all salivary gland neoplasms. Extensive squamous metaplasia in PA can be mistaken for malignancy, including low grade mucoepidermoid carcinoma and squamous cell carcinoma. Here, we present an unusual case of PA with extensive squamous metaplasia and keratin cyst formations in a minor salivary gland, and discuss its microscopic features, including the immunohistochemical characteristics, and differential diagnosis of this uncommon presentation.

Extracranial Schwannomas of the Head and Neck: A Literature Review and Audit of Diagnosed Cases Over a Period of Eight Years.

Schwannoma is a benign, slow growing, usually solitary and encapsulated tumor derived from Schwann cells of the nerve sheath. Schwannomas can be divided into central, or intraosseous, and peripheral lesions. The etiology is unknown, but it is postulated that lesions arise by the proliferation of Schwann cells at one point inside the perineurium. Schwannomas may mimic other diseases of the head and neck, such as infection, tumor or metastasis. Extracranial schwannomas are rare; in this study we review a series of 22 cases of schwannomas originating in the head and neck region over a period of eight years. All tumors were benign and well-encapsulated. Tumor size ranged from 0.5 to 9 cm. The age range of patients studied was 15-74 years with a mean age of 35 years and a male predilection (M:F, 2.6:1)was noted. Four cases of schwannomas occurred in the tongue (18.18%) and lower lip (18.18%), three in the nasal cavity (13.64%), two each (9.09%) in the buccal mucosa, parapharyngeal space (9.09%), and eyebrow (9.09%), and one each in the upper lip (4.55%), lateral canthus of the eye (4.55%), intraorbital region (4.55%), submandibular gland (4.55%), and ear (4.55%). Schwannomas can present in a wide variety of sites within the head and neck. The tumor is benign and tend to be asymptomatic for long periods of time. Histopathology is the gold standard for diagnosis. Our study describes the clinicopathologic features of extracranial head and neck schwannomas, highlights the histopathologic features, and discusses pertinent findings with correlation to the present literature. It is important that both clinicians and pathologists be familiar with the uncommon sites of occurrence and the potential pitfalls associated with the diagnosis and management of these tumors.

Adult Left Colocolic Intussusception Successfully Managed by Left Hemicolectomy and Primary Anastomosis.

Intussusception, although quite common in children with the classic triad of cramping abdominal pain, bloody diarrhea, and palpable masses, is a rare cause of acute abdomen with myriad presentations in adults. It is defined as the telescoping of a proximal segment of the gastrointestinal (GI) tract, called the intussusceptum, into the lumen of the adjacent distal segment of the GI tract, called intussuscipiens. Due to its different manifestations and time course, adult colonic intussusception often poses a diagnostic challenge for emergency doctors. The treatment of colonic intussusception in adults typically involves surgery, often with bowel resection and anastomosis followed by a defunctioning loop ileostomy. We report a case of left-sided colocolic intussusception secondary to a tubular adenoma as the lead point, which was successfully treated by resection and primary anastomosis. The pathological diagnosis of the lesion was reported as adenocarcinoma and resected bowel margins were found free of the tumor.

Microvillous inclusion disease as a cause of severe congenital diarrhea in a newborn.

Microvillous inclusion disease (MVID), also known as congenital microvillus atrophy remains an important differential diagnosis of intractable secretory diarrhea in neonatal period. The condition is inherited as an autosomal recessive disorder with no sex predilection and more commonly reported in those tribes with consanguineous marriages. The pathognomonic electron microscopic findings includes villous atrophy with the formation of intracellular microvillous inclusions. Definite treatment includes either isolated small bowel or combined small bowel and liver transplantation. Herein, we are describing a case of intractable diarrhea in a preterm neonate with MVID phenotype presented on second day of life with intractable diarrhea. The diagnosis was established by classical electron microscopic findings in the intestinal biopsy sample.