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.

Androgen Receptor Expression in Endometrial Stromal Sarcoma: Correlation With Clinicopathologic Features.

Endometrial stromal sarcoma (ESS) is a rare neoplasm comprising only 0.2% to 1% of all uterine malignancies and occurs in women between 42 and 59 yr of age. ESSs frequently express estrogen receptor (ER) and progesterone receptor (PR). However, the published literature contains scant data on the expression and therapeutic/prognostic role of androgen receptor (AR) in ESSs. We undertook this study to characterize the expression of AR along with ER and PR in ESSs and correlate it with clinicopathologic features. The clinical details, slides, and blocks of 25 tumors from 24 patients (September 2010 to February 2016) were retrieved. The diagnosis and grade of ESS were reviewed and immunohistochemistry performed with anti-ER, PR, and AR antibodies. Ages ranged from 18 to 50 yr, with a mean age of 36 yr. Low-grade ESS (LGESS) and high-grade ESS (HGESS) were diagnosed in 15 and 9 patients, respectively. An 18-yr-old woman who initially had LGESS suffered a pelvic recurrence; that exhibited high-grade morphology. Our patients, especially those with HGESS, were much younger compared with published worldwide data. ER, PR, and AR immunoreactivity was observed in 14 (93.3%), 12 (80%), and 11 (73.3%) LGESSs, respectively. This is in contrast to HGESSs, in which 5 (50%) tumors had a triple-negative hormonal profile. AR, like ER and PR, was more frequently expressed in LGESS as compared with HGESS. Whether AR, in addition to ER and PR receptor status, may help guide adjuvant hormonal therapy needs further elucidation.

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.

Cytologic and Clinicopathologic Features of Papillary Thyroid Carcinoma with Prominent Hobnail Features on FNAC.

The hobnail variation of papillary thyroid cancer (PTC) is an uncommon, aggressive variety with hobnail characteristics on more than 30% of the tumour cells. Because of the entity's rarity, the clinical behaviour and pathologic characteristics of these tumours are still unknown. The purpose of this case study was to look at the cytologic, clinical, and pathological aspects of the hobnail variant of PTC, because of its severe clinicopathologic features and poor outcome, the unusual hobnail type of PTC may necessitate more aggressive treatment than traditional PTCs. The cytologic features of the hobnail variety are distinctive, and FNAC specimens can be used to provide a preoperative diagnosis. Diagnostic parameters for proportion of hobnail morphology need to be refined further. More research is needed to understand how detection of this high-risk variation affects clinical treatment.

Uterine preservation in low-grade endometrial stromal sarcoma.

Data on uterine preservation in the management of low grade endometrial stromal sarcoma (LGESS) is scarce due to rarity of this tumor type. Standard management of LGESS involves extrafascial hysterectomy with bilateral salpingo-oophorectomy with debulking of any extrauterine metastatic disease. High estrogen and progesterone receptor expression facilitates adjuvant hormone therapy post-surgery. LGESS frequently affects young women, thus fertility preservation is an important issue in management. Here we describe uterine preservation in two young women diagnosed with LGESS followed by GnRH analogue therapy with favorable outcome. The first case was diagnosed with recurrent endometrial polyp invading myometrium requiring wedge resection of uterus with free margins. Second case presented with a vaginal mass arising from cervix and excision was done through vaginal route. Both patients were prescribed GnRH analogue therapy for six months post-surgery and are currently on follow-up. These case reports add to literature on feasibility of uterine preservation in the management of LGESS.

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.

Sudden Death Caused by Gastroesophageal Varices Rupture: Insights From an Autopsy-Based Case Series Unraveling the Pathological Events.

Sudden death is characterized by natural yet unexpected death, typically occurring within 24 hours from the onset of the patient's symptoms. While the majority of sudden deaths stem from cardiac issues/causes, there are instances where non-cardiac factors are at play. One such scenario involves hemorrhage from ruptured esophageal varices, a complication that stems from portal hypertension. Portal hypertension can manifest due to a range of pre-hepatic, hepatic, and post-hepatic conditions, with liver cirrhosis being the primary culprit. Although sudden death cases linked to the gastrointestinal system are relatively rare, the rupture of gastroesophageal varices, precipitating severe morbidity and a high mortality rate, represents a life-threatening condition. In this context, we present a case series encompassing five instances of sudden natural deaths arising from the rupture of gastroesophageal varices.

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.

Epidermoid Cyst: An Esthetic Challenge, If Neglected-A Comprehensive Analysis of 217 Cases.

Background: An epidermoid cyst is a benign keratin-filled cyst, commonly located in the face, scalp, neck, and trunk. Although it is a common benign cystic neoplasm, large series of analyses in English literature are still scarce. To comprehend the clinical and pathological variation of epidermoid cysts in the sub-Himalayan region of India, as well as their clinical implications for late diagnosis, a retrospective study on epidermoid cysts was conducted. Materials and Methods: This was a retrospective observational study conducted on 217 cases of epidermoid cyst subjected for fine-needle aspiration cytology over a period of 3.6 years (from 2018 to 2021). The clinical details and other epidemiological data were archived from the pathology requisition forms. Results: It showed slight male preponderance with head and neck site as the most common area. Most patients were adults. Few uncommon sites were also seen namely breast, parotid, epididymis, and tonsils. Few large lesions were identified measuring up to 10 cm. The skin changes were seen in 5.5% cases, 0.9% cases show extreme ulceration, and 4.1% cases show foreign body giant cell reactions. Due to the province's steep terrain and limited access to medical services, the majority of patients arrived at out patient department (OPD) late. Conclusion: The findings suggest that fine-needle aspiration can be used as a quick diagnostic test even in remote areas by the general practitioner who can send the slides for evaluation to a pathologist. Many times, clinically looking epidermoid cyst can reveal certain challenging entities such as a biphasic fibroepithelial tumor with cystic squamous metaplasia and proliferating pilar tumor in our series. The study of this lesion is also needed for early diagnosis as long-standing lesion may rupture and cause requirement of extensive treatment followed cosmetic impairment.

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.

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).