Hobnail subtype of papillary carcinoma of the thyroid-a rare case with uncommon features.

The cytological features of the hobnail variant of papillary thyroid carcinoma may be subtle. It is important to recognize this variant because it may influence the corresponding surgical treatment and follow-up due to its aggressive nature. The hobnail subtype of papillary thyroid carcinoma is a rare entity with aggressive features. It presents extrathyroidal extension or lymph nodal metastasis in a high percentage of the cases.

Neonatal autopsy-is it relevant in today's era?

Autopsy of infants can provide vital information about the cause of death and contributes to the detection of diagnostic errors, especially in a low- or middle-income country. To observe the clinicopathological agreement in neonatal deaths in neonatal intensive care units (NICU) and comment on the additional information retrieved by autopsy. A retrospective observational study was conducted in the NICU from January 2020 to December 2022. Neonatal deaths were analyzed, and clinical details and autopsy findings were collected. Both clinical and pathological diagnoses were classified according to the Goldman classification. Twenty-two newborn infants were enrolled. The mean gestational age was 33.5 (±4.38) weeks, and the median birth weight was 1510 (1005-2100) g. There was complete concordance between clinical and pathological diagnosis in 11 (50%) cases. Major diagnostic errors occurred in 41% of cases. Respiratory system disorders (lung infections, airway anomalies) accounted for six (54%) cases of missed diagnosis. Our study showed that the diagnosis was revised after autopsy in about one-third of cases, and newer findings were identified in one-fifth of cases.

Congenital Intraoral Dermoid and Epidermoid Cyst with Orocutaneous Fistula Presenting with Life-threatening Airway Obstruction in an Infant.

Dermoid cysts and epidermoid cysts in the floor of the mouth are rare in the pediatric age group. In this case report, we have discussed the presentation, management, and literature review of a 6-month-old female child presenting with both dermoid and epidermoid cysts in the floor of the mouth with an orocutaneous fistula.

Holocord metastasis from supratentorial glioblastoma multiforme: an uncommon manifestation of a common tumor.

Intramedullary metastasis from primary glioblastoma multiforme (GBM) is a rare phenomenon with a poor prognosis. The rate of spinal metastasis from intracranial GBM has been variably reported to be 0.4-2%. According to a review by Lawton in 2012, there were only 42 documented cases of primary intracranial GBM with spinal metastasis. We present a unique case of early-onset symptomatic holocord metastasis of GBM in a patient approximately 2 months of detection of primary GBM.

Prevalence of Pancreato-Biliary Maljunction in Children with Choledochal Cyst - A Prospective Observational Study.

Background: Pancreatico-biliary maljunction (PBM) is often found to be associated with choledochal cysts (CCs). According to a European multicenter study, the prevalence of PBM in cases of CC is found to be 72.2%, however, there is no Indian study depicting the prevalence of PBM in Indian children with CCs, which is one of the main postulated factors in the etiopathogenesis of CC. In this study, we have attempted to observe prospectively the prevalence of PBM in children with CC and correlate this to its morphological and biochemical parameters. The association between the presence of PBM and histopathological findings such as epithelial changes of the mucosa of the CC, inflammation, metaplasia or dysplasia, and histopathology of the liver has also been evaluated. Materials and Methods: We carried out a single center, prospective observational study with a single arm study group. We prospectively selected all patients of CC admitted for surgery from November 2018 to October 2020. Data on biochemical, radiological, and histopathological parameters were collected and analyzed. Results: We included a total of 20 patients in our study. The mean age of the participants was 6.22 ± 4.32 years. Among them, 11 (55.0%) were male and 9 (45%) were female. Abdominal pain was the most common presenting complaint among our patients (75.0%) and had a significant association with the presence of a PBM (P = 0.001). In symptomatic children, the mean duration of symptoms was 4.50 ± 2.26 months for jaundice, 4.50 ± 1.98 months for abdominal distension, and 5.07 ± 2.02 months for abdominal pain. Among the 3 children with cholangitis, the mean number of episodes was 3.33 ± 2.08, with a median of four episodes. Fourteen (70.0%) of the children had type I a CC, 1 (5.0%) participant each for types I b, I c, II and IV a and 2 (10.0%) of them had type IV b cyst. The mean size of the cyst (cm) was 7.41 ± 3.03 with a median of 6.85 cm. Among the children, 9 (45%) showed the presence of PBM on magnetic resonance cholangiopancreatography (MRCP), with 7 (77.8%) showing Komi's C-P type and 2 (22.2%) showing Komi's PC type. The mean common channel length (mm) on MRCP was 8.11 ± 2.47 with a median length of 8.00 mm. The biochemical analysis of a bile fluid amylase and lipase is the functional indicator of the presence of a PBM. Histopathological examination showed the presence of ulceration in the walls of the CC in 10 (50.0%) of the specimens. There was a significant association of the presence of PBM and ulceration in the mucosa of the CC (P ≤ 0.001), with the median levels being the highest in the PBM present group. Conclusion: Abdominal pain is the most common complaint in a child with CC, and when present, it is significantly associated with the presence of a PBM. MRCP is the gold standard tool to detect CCs and find out the morphology of PBM. The prevalence of PBM in children with CC of 45% with a mean common channel length of 8.11 mm. The biochemical analysis of a bile amylase and lipase is the functional indicator of the presence of a PBM and there is a significant association of their higher levels and presence of PBM. The presence of chronic inflammation and microscopic ulcers is significant histologic parameters depicting the presence of a PBM.

Biliary Cystadenoma in a Child: A Rare Entity.

Biliary cystadenoma, a rare potentially malignant hepatic cystic lesion, is characterized by multiloculations and septations. It is common in middle-aged females (about 5% of nonparasitic liver cysts); only 12 cases are described in children. We report a rare case of hepatic biliary cystadenoma in a 3-year-old girl, with a gradually increasing lump in the right upper abdomen. Complete excision with a healthy liver margin was done.

Aesthetic Outcome of Gynecomastia Management with Conventional Liposuction and Cross-Chest Liposuction: A Prospective Comparative Study.

BACKGROUND: Liposuction is the most accepted technique for treatment of gynecomastia at present with or without residual gland tissue excision. Conventional liposuction uses incisions for introduction of cannula, made usually at the inframammary crease or axilla resulting in consequent scars. Cross-chest liposuction technique was introduced to avoid these additional scars and improve the aesthetic outcome. This study aimed to evaluate the difference between aesthetic outcome of conventional liposuction and cross-chest liposuction in treatment of gynecomastia. METHOD: A prospective comparative study between 2 groups with 15 patients in each was conducted, one with conventional and the other cross-chest liposuction. Excision of residual gland tissue was performed through circum-areolar incision in both the groups. Aesthetic outcome was evaluated in both the groups using Likert scale and compared. RESULT: The mean age of the patients was 22.6 years and the mean duration of presentation was 8.13 years. Satisfaction rate with conventional and cross-chest liposuction was 80% and 86%, respectively, as assessed by the patients. The rate was 80% and 77%, respectively, in both the groups as assessed by independent observer. The complication rate was 13.3% in conventional and 20% in cross-chest group. The difference in outcome was not statistically significant between two groups. Difference between the mean Likert scores of pre-operative and post-operative satisfaction was statistically significant for both the techniques. CONCLUSION: The conventional and the cross-chest liposuction, both yield comparable results when used for treatment of gynecomastia in terms of aesthetic outcome. LEVEL OF EVIDENCE III: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266.

Rectal Inflammatory Myoglandular Polyp with Osseous Metaplasia in a Child.

Background: Inflammatory myoglandular polyp (IMGP) combines the histopathological features of juvenile polyp and Peutz-Jegher's polyp. Osseous metaplasia can be occasionally seen in various benign, adenomatous, and malignant polyps. Case report: A nine-year-old girl with hematochezia had a rectal polyp with histological features of IMGP with additional stromal osseous metaplasia. Conclusion: Benign osseous metaplasia can be found in IMGP.

Factors predicting perioperative outcomes in patients with myasthenia gravis or thymic neoplasms undergoing thymectomy by video-assisted thoracoscopic approach.

BACKGROUND: The purpose of this study was to identify the factors which predict the perioperative outcomes after video-assisted thoracoscopic surgery (VATS) thymectomy in patients with myasthenia gravis (MG) or thymic neoplasms. PATIENTS AND METHODS: Data of consecutive patients who had undergone VATS thymectomy in our institution from August 2016 to April 2018 were collected retrospectively from a prospectively maintained database followed by prospective recruitment of patients who underwent VATS thymectomy from April 2018 to February 2020. RESULTS: A total of 31 patients were included. Females were more common (51.6%), and 29 patients (93.5%) had MG. The most common indication for thymectomy was the presence of both MG and thymoma (51.6%). Most MG patients had moderate disease (55.2%) or severe (24.1%) disease preoperatively. Mean operative time and blood loss were 196.9 ± 63.5 min and 122.5 ± 115.3 ml, respectively. Mean hospital stay was 7.9 ± 6.7 days. The rate of major and minor complications was 16.18% and 35.4%, respectively. Multivariate linear regression analysis established that MG symptoms >12 months, prolonged invasive ventilation (intubation ≥24 h), and complications were associated significantly with a prolonged hospital stay. Adjusting for outliers, pre-operative disease severity (MG Foundation of America class), and intubation ≥24 h were the only factors that had a significant impact on perioperative outcomes. CONCLUSION: Pre-operative disease severity and post-operative invasive ventilation are strong determinants of perioperative outcomes. Pre-operative optimisation and early extubation protocols can further reduce morbidity in patients undergoing thymectomy by the VATS approach.

Xanthogranulomatous cystitis masquerading as bladder tumor in a child.

Xanthogranulomatous cystitis affecting the urinary bladder is extremely rare, and only around thirty adult cases and two pediatric cases have been reported in the literature. The treatment is predominantly surgical as the lesion is mostly infiltrative and mimics malignancy. We report probably the third pediatric case, who presented with symptoms of urinary tract infection and urinary retention and was initially suspected as bladder tumor on imaging. The diagnosis was confirmed on histopathology, and the child responded well to aggressive antibiotic therapy alone.

Spinal hemangiopericytoma: an institutional experience and review of literature.

PURPOSE: Hemangiopericytoma is a rare tumor of CNS with potential for recurrence and widespread metastasis, even outside CNS with even rare involvement of spinal cord. This case series presents five patients to evaluate the clinical presentation, radiological features, management, pathology and outcome of spinal hemangiopericytomas. METHODS: Between 2004 and 2013, five patients underwent surgery for spinal hemangiopericytoma. Histopathological data were reviewed in all cases and clinical and follow-up details were collected from the data available in our department. RESULTS: There were three males and two females, including one pediatric patient. Three patients had dorsal spine involvement and two patients had involvement of cervical spine. There were two patients with intradural extramedullary tumors, one patient each with pure intramedullary tumor, pure extradural tumor and both intra and extradural tumor. All of them presented with motor weakness. Gross total resection of the tumor was done in three patients. Four patients received post-operative radiotherapy. Histopathology showed anaplastic tumor in four cases with high MIB-1 LI. Most of them were positive for CD34, mic-2 and bcl-2. Three patients who underwent gross total resection improved significantly in the follow-up period. Two patients who underwent subtotal resection expired due to spread of their disease. CONCLUSION: Spinal hemangiopericytoma is a rare tumor. Strong clinical suspicion is required to diagnose it pre-operatively. Gross total resection is the goal and radiotherapy should be given in case of residual tumor or high-grade tumors.

Cerebellar Liponeurocytoma, an Unusual Tumor of the Central Nervous System--Ultrastructural Examination.

Cerebellar liponeurocytoma is a rare tumor of the central nervous system which shows neuronal and variable astrocytic differentiation, along with foci of lipomatous differentiation. It is usually located in the cerebellum, and may be mistaken for medulloblastoma with lipidized cells or lipomatous ependymoma. Histopathological examination, supplemented by immunohistochemistry and electron microscopy, is required to distinguish between these entities. This 35-year-old male presented with vomiting and headache for three months, followed by gait imbalance. Neurological examination showed positive cerebellar signs with ataxic gait. Magnetic resonance imaging showed a lesion measuring 4.4 cm× 4.3 cm× 3.9 cm involving the cerebellum. The patient underwent midline suboccipital craniotomy to excise the tumor. Histopathological examination showed a circumscribed, cellular tumor composed of round to polygonal cells with moderate cytoplasm and minimal pleomorphism. Clear intracytoplasmic vacuoles were seen within the tumor cells. These tumor cells were immunopositive for synaptophysin, NSE, and MAP-2, confirming their neurocytic origin. On ultrastructural examination, lipid vacuoles as well as dense-core neurosecretory granules were identified within these neurocytic cells, confirming the diagnosis of liponeurocytoma. No cilia, microvilli, or gap junctions were identified in the tumor cells, ruling out the possibility of lipomatous ependymoma. The differentiation of liponeurocytoma from its morphological mimics is imperative, as their treatment differs drastically. The role of electron microscopy is extremely important in this differential diagnosis.

Epithelioid hemangioma of distal femoral epiphysis in a patient with congenital talipes equinovarus.

BACKGROUND: Epithelioid hemangioma (EH) is a rare benign vascular lesion of soft tissue and bone, characterized by endothelial cells with epithelioid or histiocytoid appearance. Though tubular bones, flat bones, vertebra and short bones are common sites for this lesion, the epiphyseal involvement is extremely rare. We present an unusual case of EH of the distal femur in a young boy. CASE REPORT: A 12-year-old boy who had congenital talipes equinovarus of the right foot presented with progressively increasing pain in the right lower thigh for six months. Physical examination revealed muscular atrophy of the right lower limb and a moderately tender swelling in the medial aspect of the right knee without restriction of knee movement. An X-ray revealed an osteolytic lesion, which appeared iso- and hypointense on T1W and hyperintense on T2W MRI images in the distal epiphysis and adjacent metaphysis of the right femur. A radiological diagnosis of chondroblastoma was entertained. The patient was treated with curettage and bone grafting. Histopathology showed a tumor composed of thin-walled arteriolar capillaries lined by large, polyhedral epithelioid endothelial cells with vesicular nuclei, finely distributed nuclear chromatin, and moderate amount of eosinophilic cytoplasm. The endothelial cells were strongly immunopositive for CD34. Mitotic activity was low and the Ki-67 proliferative rate was <2%. A diagnosis of EH was made. EH is a benign lesion and it should be differentiated from its histologically similar malignant counterparts such as epithelioid hemangioendothelioma and epithelioid angiosarcoma as the lesion can be successfully treated with curettage or resection.

Determination of target genes for classified molecular subtypes of triple-negative breast cancer form microarray gene expression profiling: An integrative in silico approach.

BACKGROUND: Highly heterogeneous triple-negative breast cancer (TNBC) has tough clinical features, which were gradually solving and improving in diagnosis by the molecular subtyping of TNBC. AIM: Presently, this study was focused on analyzing the genetic makeup of TNBC subtypes. SETTINGS AND DESIGN: This study explored the MicroArray expression profiling of differentially expressed genes in molecular subtypes BL1, BL2, IM, luminal androgen receptor, M, and mesenchymal stem-like of TNBC by analyzing the Gene Expression Omnibus dataset GSE167213. Various gene ontologies-based protein-protein interaction (PPI) networks were subtyped TNBC genes. The effect of genetic alteration on TNBC cases was also interpreted. MATERIALS AND METHODS: The MicroArray gene expression profiling was done through R programming and subjected to functional annotation through the database for annotation, visualization, and integrated discovery. The PPI networking of functionally associated genes was interpreted by STRING. The survival analysis was done through cBioPortal. STATISTICAL ANALYSIS USED: The t -test was used through R programming to generate the P values for a test of the significance of expressed genes. RESULTS: A total of 54,613 significant probes were analyzed in the TNBC MicroArray dataset. The functional PPI networks of BL1, BL2, and IM upregulated genes showed significant associations. The survival analysis of differentially expressed genes showed the significant prognostic effect of 32 upregulated genes of different subtypes on TNBC cases with genetic alterations, whereas the remaining genes showed no significant effects. CONCLUSION: The output of the present study provided significant target gene panels for different TNBC subtypes, which would add an informative genetic value to TNBC diagnosis.

Syringoid eccrine carcinoma of the nipple in a male patient with lymph node metastasis: A case report.

ABSTRACT: Syringoid eccrine carcinoma of nipple is an extremely rare neoplasm of adnexal origin with variable clinical appearance and diverse histologic findings. Syringoid eccrine carcinoma (SEC) is often a diagnostic dilemma due to its morphology and presentation. Usually, these malignancies arise as non-ulcerated nodules or plaques in the head & neck region including the trunk. They are locally aggressive and have an infiltrative growth pattern with a propensity for metastasis. SEC is characterized by syringoma-like tadpole morphology with ductular differentiation and predominant desmoplasia. Immunostaining in SEC is variable and this variability is believed to arise from the tumor's ability to differentiate along multiple routes including sweat secretory and or ductal differentiation. Here we present a rare case of SEC/ syringomatous carcinoma of nipple in a 51-year-old male breast with associated axillary lymph node metastasis. As per English literature, this is the second case of SEC in nipple of male patient.

An unreported case of intraoperative frozen section diagnosis of a sclerosing stromal tumor of the ovary- A report with a review.

ABSTRACT: Sclerosing stromal tumor is a benign sex cord-stromal tumor, that commonly occurs in the second and third decades of age. Intraoperative diagnosis of this entity poses a great challenge because of the rare occurrence and can mimic malignant lesions. A 15-year-old female presented with a right ovarian mass. Serum markers were within normal limits. The radiological evaluation showed a large heterogeneously enhancing solid cystic abdominopelvic mass of size 16 × 14 × 9 cm with non-visualization of both ovaries separately and a few areas of calcification with mild ascites. An open cystectomy was performed. A part of the cyst wall was sent for an intraoperative frozen section. It was reported as sclerosing stromal tumor, and the post-operative specimen also confirmed the same. Areas of calcification and ossification were also identified as additional findings. We reported this case because of the uncommon occurrence, highlighting additional histological features, and also did a literature review, especially focussing on the intra-operative diagnosis.

Currarino syndrome with immature teratoma: A case report with review of literature.

ABSTRACT: Currarino syndrome (CS) is a rare congenital syndrome characterized by a triad of anorectal malformation, sacral deformity, and presacral mass. In about 50% of cases, it is caused by HLXB9 gene mutation in chromosome 7q36. A 13-month-male child presented with presacral discharging sinus with a history of surgery for anorectal malformation and perineal fistula at the time of birth. On detailed investigation, the child revealed to have anal atresia, hemisacrum, and presacral mass. Histopathology of presacral mass showed features of immature teratoma. The presacral mass in CS is mostly an anterior myelomeningocele or presacral teratoma. The development of immature teratoma in presacral mass is very rare. The histopathological identification of immature component of teratoma in the presacral mass of CS is important for risk stratification and further management. Suspicion of CS should be raised in any child presenting with partial phenotype of the triad.

Primary invasive squamous cell carcinoma of the nipple in an adult male: A case report and brief review of literature.

This is a case report of a rare and uncommon primary invasive squamous cell carcinoma (SCC) of the nipple in a male patient. The patient presented with an ulcerated growth over the left nipple for the last 20 years, which progressed over the last 6 months. He underwent wide local excision with level II axillary lymph node dissection, and one out of 42 lymph nodes harvested showed metastatic deposit. The patient was planned for follow-up with no adjuvant treatment, and had no evidence of local-regional or distant recurrence at 24 months follow-up. The primary invasive SCC of male nipple is very rare, and its diagnosis is challenging as it can be confused with other clinical conditions. However, a histopathological examination with immunohistochemistry can differentiate primary SCC nipple from other differential diagnoses. The treatment options for cutaneous SCC include surgical excision, cryotherapy, electrosurgery, topical ointments, definitive radiation therapy, and photodynamic therapy. Regional lymph node dissection in SCC nipple could potentially have therapeutic and prognostic significance.

A Novel Report of mycetoma with Spinal Spread due to Madurella fahalli from India.

Eumycetoma caused by Madurella fahalii, a drug-resistant fungus, has never been reported in India. Here, we describe a fatal case of eumycetoma with spinal involvement due to M. fahalii for the first time in India.

Acceptance of emerging renal oncocytic neoplasms: a survey of urologic pathologists.

Oncocytic renal neoplasms are a major source of diagnostic challenge in genitourinary pathology; however, they are typically nonaggressive in general, raising the question of whether distinguishing different subtypes, including emerging entities, is necessary. Emerging entities recently described include eosinophilic solid and cystic renal cell carcinoma (ESC RCC), low-grade oncocytic tumor (LOT), eosinophilic vacuolated tumor (EVT), and papillary renal neoplasm with reverse polarity (PRNRP). A survey was shared among 65 urologic pathologists using SurveyMonkey.com (Survey Monkey, Santa Clara, CA, USA). De-identified and anonymized respondent data were analyzed. Sixty-three participants completed the survey and contributed to the study. Participants were from Asia (n = 21; 35%), North America (n = 31; 52%), Europe (n = 6; 10%), and Australia (n = 2; 3%). Half encounter oncocytic renal neoplasms that are difficult to classify monthly or more frequently. Most (70%) indicated that there is enough evidence to consider ESC RCC as a distinct entity now, whereas there was less certainty for LOT (27%), EVT (29%), and PRNRP (37%). However, when combining the responses for sufficient evidence currently and likely in the future, LOT and EVT yielded > 70% and > 60% for PRNRP. Most (60%) would not render an outright diagnosis of oncocytoma on needle core biopsy. There was a dichotomy in the routine use of immunohistochemistry (IHC) in the evaluation of oncocytoma (yes = 52%; no = 48%). The most utilized IHC markers included keratin 7 and 20, KIT, AMACR, PAX8, CA9, melan A, succinate dehydrogenase (SDH)B, and fumarate hydratase (FH). Genetic techniques used included TSC1/TSC2/MTOR (67%) or TFE3 (74%) genes and pathways; however, the majority reported using these very rarely. Only 40% have encountered low-grade oncocytic renal neoplasms that are deficient for FH. Increasing experience with the spectrum of oncocytic renal neoplasms will likely yield further insights into the most appropriate work-up, classification, and clinical management for these entities.