Clinicopathological predictors of repeated recurrence in sinonasal inverted papilloma.

PURPOSE: To evaluate association between clinical and pathological findings and repeated recurrence in sinonasal inverted papilloma. METHODS: Retrospective cohort study conducted at a tertiary care teaching hospital included all patients operated for inverted papilloma from January 2010 to December 2019. Patients were categorized as primary and recurrent cases. Based on disease status at follow-up, they were subcategorized into 'primary with no recurrence' (PnR), 'primary with recurrence' (PwR), 'recurrent with no further recurrence' (RnR), and 'recurrent with further recurrence' (RwR) groups. Data including demography, clinical, endoscopic and pathological findings were collected and analyzed. RESULTS: Increased incidence of pale appearance of lesion in RnR group (p = 0.017), polypoidal appearance in primary group (p = 0.002) and fibrous appearance in the recurrent group (p = 0.002) were statistically significant. Predominant epithelium was combined respiratory and squamous epithelium in primary and recurrent groups and also in RnR group (p = 0.019), while it was squamous (p = 0.024) in RwR group. Epithelial hyperplasia was more common in primary and RnR groups. Oncocytic change, cystic dilatation, microabscess and squamous metaplasia were seen more in recurrent and RnR groups. Cytoplasmic glycogenation was more in recurrent and RwR groups. Stroma was predominantly edematous in all the groups. CONCLUSIONS: Patients with recurrence are younger and present earlier than those with primary disease. Fleshy appearance and pink/red colour of tumour, lining epithelium being squamous and cytoplasmic glycogenation could be considered as features predicting recurrence. Negative predictors of recurrence of IP include pale appearance of tumour, combined respiratory and squamous epithelium lining and squamous metaplasia.

Study of pathogenic T-helper cell subsets in Asian Indian patients with Takayasu arteritis.

The relapses and refractory disease are a challenge in the management of patients with Takayasu arteritis (TAK). We quantified pathogenic CD4 + memory T helper cells bearing surface markers CD161 and/or p-glycoprotein (MDR1) in patients with TAK. Peripheral blood mononuclear cells of 21 patients with TAK and 16 age-matched controls were stained with anti-CD3, anti-CD4, anti-CD45RA, anti-CD161 and anti-p-glycoprotein antibodies and subjected to flow cytometry by FACS ARIAIII. Eighteen patients underwent follow-up immunophenotyping. Intracellular staining for interleukin-17 and interferon-γ was performed for 18 patients and 11 controls. Surgical arterial biopsies of 6 TAK and 5 non-inflammatory controls were subjected to immunohistochemistry with anti-CD161 and anti-p-glycoprotein. At baseline the frequency of MDR1 + CD4 + and CD161 + MDR1 + CD4 + memory T cells was higher in TAK than controls (p = 0.002 and 0.01, respectively). After stimulation, the frequency of IFN-y + CD161 + cells was higher in TAK than controls (p = 0.028). Modal fluorescence intensity of CD161 + MDR1 + CD45RA - CD4 + cells was higher in active as compared with stable disease (p = 0.041). At 6 months, MDR1 + and CD161 + MDR1 + memory CD4 + T cells decreased significantly only in patients who had complete/partial response to treatment (p = 0.047 and 0.02, respectively). To conclude, MDR1 + and MDR1 + CD161 + CD4 + memory T-helper cells are increased in patients with TAK. These cells decreased only in patients with response to treatment during subsequent follow-up.

A Rare Presentation of Recurrent Malignant Peripheral Nerve Sheath Tumor with Glandular Differentiation-A Case Report.

Malignant peripheral nerve sheath tumors (MPNST) are rare heterogeneous group of soft tissue neoplasms. In most cases, they originate within the pre-existing neurofibromatosis. The emergence of glandular structures in MPNST is curious and enigmatic. We report a case of recurrent MPNST with glandular differentiation arising in the background of neurofibroma in a 20-year-old lady. By immunohistochemistry, MPNST showed focal positive staining for S100 and negative staining for SOX10 while adjacent neurofibroma showed diffuse positivity for S100 and SOX10. The glandular tumor cells showed positive staining for CDX2, Cam5.2, CK19, and CK7 (focal), while negative for SOX10 and S100. MPNST with glandular differentiation is quite rare which may pose a diagnostic challenge. The glandular differentiation in MPNST should be excluded from the metastasis from second primary with the aid of clinical and radiological correlation.

Malignant Gastrointestinal Neuroectodermal Tumor of Small Intestine Showing DOG1 Expression: A Case Report and Review of Literature.

Malignant gastrointestinal neuroectodermal tumor (GNET), also referred to as clear cell sarcoma-like tumor of the GI tract is a rare mesenchymal tumor of the gastrointestinal tract. It has to be distinguished from various mimickers including gastrointestinal stromal tumor (GIST) due to its aggressive course and different natural history and therapeutic approach. Here we report a case of GNET arising in the small intestine with aberrant DOG1 expression posing a diagnostic challenge. In this context, the combination of clinical, histomorphological, immunohistochemical, and molecular features helped to establish a proper diagnosis.

Signet ring cell carcinoma of the rectal stump in a known ulcerative colitis patient.

Colorectal carcinoma (CRC) is the third most commonly diagnosed cancer worldwide and is the second most common cause of cancer-related deaths. However, the Omani population shares the major burden as the most prevalent carcinoma. The disease is comparatively higher in males than females. Patients with pre-existing risk factors, including inflammatory bowel disease, are at increased risk of developing neoplasia. Among the various histopathological subtypes of adenocarcinoma in the rectum, signet ring cell carcinoma is the rarest and accounts for approximately 1% of the cases. Given the aggressive nature of this tumor, advanced presentation, stage, and poor prognosis, regular endoscopic surveillance is essential. Hereby, we report a rare case of signet ring cell carcinoma arising in the rectal stump in an already diagnosed and operated patient of Ulcerative colitis.

Frontoethmoidal Extranasopharyngeal Angiofibroma With Orbital Pyocele.

Significance StatementExtranasopharyngeal angiofibromas (ENA) are rare vascular tumors that do not conform to the clinical characteristics of typical nasopharyngeal angiofibromas. We present the management of an angiofibroma in a rare site, within the frontal sinus with a concomitant orbital pyocele, which was completely excised via an endoscopic approach. ENAs should be considered as a differential diagnosis in patients with sinonasal mass and epistaxis. Awareness of this rare entity will avoid radical surgery thus decreasing postoperative morbidity.

Myoepithelial Tumors of Salivary Gland: A Clinicopathologic and Immunohistochemical Study of 15 Patients with MIB-1 Correlation.

Myoepithelial neoplasms are rare tumors of the salivary glands with predominant myoepithelial differentiation and a broad histologic spectrum. Their histological features, immunohistochemical profile and biological behavior are not well characterized and pose a diagnostic challenge. A total of 15 myoepithelial tumors, diagnosed during 2012 and 2019 were subcategorized and correlated with MIB-1 labeling index (LI) and various histological parameters. Immunohistochemical stains for MIB-1 and other antibodies were performed. Statistical analysis was done by chi-square test, Fisher's exact test and Kaplan Meier curve. Nine patients were male and six were female with the median age of 44 years (range 21-83 years). Of the 15 patients, 6 cases were classified as myoepithelioma (ME) and 9 cases as myoepithelial carcinoma (MECA). Parotid gland was the most common site (46.7%) followed by the palate. MEs showed well circumscribed tumor borders whereas MECAs exhibited focal capsular to extensive invasion into adjacent tissues. Epithelioid cell morphology was most common followed by mixed cell morphology. MIB-1 LI was significantly associated with invasive tumor borders, necrosis and high mitosis. Increased frequency of recurrence was noted with high MIB-1 LI, though it was not statistically significant. MIB-1 LI was high in nearly all MECAs with focal capsular to extensive invasion while low in MEs. Myoepithelial tumor with multinodular growth pattern and focal capsular invasion may have an indolent behavior if mitotic activity and MIB-1 LI is low. Early diagnosis and treatment of MECAs significantly improves the patient's survival and prognosis.

Disseminated mycobacterial septicemia presented as acute abdomen: a surgeon's perspective on Landouzy's sepsis.

A 20-year-old man presented in emergency with fever, abdominal pain and obstipation. On evaluation, he was found to have an acute abdomen with septic shock. The cross-sectional abdominal imaging revealed hepatosplenomegaly, pleural effusion and ascites with retroperitoneal lymphadenopathy. He was resuscitated and started on broad-spectrum antibiotics. There was no other source of infection identified elsewhere. While bacterial and fungal cultures were negative, the sputum, blood, bone marrow and ascitic fluid were positive for Mycobacterium tuberculosis following which he was started on antituberculosis therapy. Despite therapy, the patient's clinical condition continued to deteriorate requiring critical care. In view of Landouzy's sepsis, pulse steroid therapy was started. However, the patient's clinical condition continued to deteriorate and developed systemic inflammatory response syndrome and multi-organ dysfunction syndrome. Despite the best efforts, the patient expired.

Management of the Thyroid Gland in Papillary Carcinoma of the Thyroglossal Cyst: A Case Report.

Carcinoma arising in a thyroglossal cyst is rare. We present a case of anterior neck swelling diagnosed to be thyroglossal cyst clinically which turns out to be a papillary carcinoma arising in thyroglossal cyst. She underwent sistrunk procedure with total thyroidectomy and diseasefree on follow up evaluation. Even though preoperative ultrasonography had shown thyroid nodule, the final histology did not show malignancy. There is a paucity of clear-cut guidelines in the management of the thyroid gland in a thyroglossal cyst carcinoma. In thyroglossal cyst carcinoma cases, we recommend thyroidectomy only when there is a thyroid nodule with high-risk features.

Estrogen and progesterone receptor in meningiomas: An immunohistochemical analysis.

BACKGROUND: Meningiomas are common slow-growing primary intracranial neoplasms attached to the dura mater and are composed of neoplastic meningothelial cells. Increased incidence of meningiomas in women with an increased tumor growth during pregnancy and a possible association with breast cancer suggested that female sex hormones have been involved in the growth of meningiomas. Antihormonal-targeted therapy would be beneficial in such patients. AIM: The aim of this study is to correlate the expression of estrogen receptor (ER) and progesterone receptor (PR) in meningiomas with gender, location, histological subtypes, and grade. MATERIALS AND METHODS: This is a 3½-year prospective and retrospective study of intracranial and intraspinal meningiomas. Clinical details of all the patients were noted from the computerized hospital information system. Immunohistochemistry for ER and PR was performed. Statistical analysis was performed using Chi-square test. RESULTS: During the study period, there were 80 Grade I, 18 Grade II, and 2 Grade III meningiomas categorized as per the World Health Organization 2007 classification. The female-to-male ratio was 1.9:1 and the mean age was 47.8 years. ER was expressed in 2% of meningiomas. PR was expressed in 67.5% of Grade I and 66.6% of Grade II and none of Grade III meningiomas. Brain-invasive meningiomas showed 54.5% PR immunopositivity and negative for ER. CONCLUSION: ER and PR were expressed in 2% and 66% of meningiomas, respectively. Statistically significant relationship was not found between the positivity of PR in females and males of Grade I and Grade II/III meningiomas, intracranial and spinal tumors, Grade I and Grade II/III cases, and various histological subtypes of meningiomas.

Ectomesenchymal chondromyxoid tumour: an uncommon characteristic tumour of the anterior tongue.

Ectomesenchymal chondromyxoid tumour (ECMT) is a rare benign tumour which classically presents in the anterior tongue. This tumour is grossly under-reported due to lack of immunohistochemical staining in many centres. We report a 46-year-old man who presented with mass in the anterior tongue and was diagnosed with ECMT. Further management of this lesion is explained with a review of the literature.

Polymorphous Cutaneous Sarcoidosis Associated with Peripheral Vascular Disease and its Dermatoscopic Findings.

Sarcoidosis is a multisystem disorder with cutaneous involvement with myriad of morphological presentations, often leading to diagnostic dilemma. We report a case of 31-year-old male with peripheral arterial disease who presented with three morphological forms of sarcoidosis simultaneously, namely, papular, psoriasiform, and pigmented purpuric dermatosis-like lesions. Dermatoscopy of cutaneous lesions showed yellow-orange globules, red dots, linear vessels, and white crystalline structures depending on the clinical forms. Histopathology of all three morphological types of skin lesions demonstrated sarcoidal naked granulomas. Sarcoid specific lesions of more than one morphological type presenting in a same patient is rare. Association of peripheral vascular disease with sarcoidosis is also seen rarely.

Signet ring cell carcinoma of the rectal stump in a known ulcerative colitis patient

ABSTRACT Colorectal carcinoma (CRC) is the third most commonly diagnosed cancer worldwide and is the second most common cause of cancer-related deaths. However, the Omani population shares the major burden as the most prevalent carcinoma. The disease is comparatively higher in males than females. Patients with pre-existing risk factors, including inflammatory bowel disease, are at increased risk of developing neoplasia. Among the various histopathological subtypes of adenocarcinoma in the rectum, signet ring cell carcinoma is the rarest and accounts for approximately 1% of the cases. Given the aggressive nature of this tumor, advanced presentation, stage, and poor prognosis, regular endoscopic surveillance is essential. Hereby, we report a rare case of signet ring cell carcinoma arising in the rectal stump in an already diagnosed and operated patient of Ulcerative colitis.

Twisted intra-abdominal cyst in a neonate: a surprise revelation.

We, herein, present a male neonate with an antenatally detected intra-abdominal cyst who presented at 18 days of life at which time, the ultrasound revealed a 5×4 cm cyst. Since he was asymptomatic, we planned to repeat the ultrasound a month later and operate if the cyst showed no regression. However, a week later, he presented with an acute abdomen, irritable cry and a repeat ultrasound showing a larger (8×6 cm) cystic mass with debris within. He was taken up for an emergency laparotomy. Intraoperatively, the cyst was found arising from the left lateral abdominal wall free from all structures with a twisted pedicle. Histopathology surprisingly revealed seminiferous tubules within the cyst wall with the vas deferens, thus confirming the diagnosis of a torsion of intra-abdominal testis. Hence, we emphasise the importance of examining for an undescended testis when dealing with a male neonate presenting with a cystic intra-abdominal mass.

Clinicopathological Study of 18 Cases of Inflammatory Myofibroblastic Tumors with Reference to ALK-1 Expression: 5-Year Experience in a Tertiary Care Center.

BACKGROUND: Inflammatory myofibroblastic tumor is a histopathologically distinctive neoplasm of children and young adults. According to World Health Organization (WHO) classification, inflammatory myofibroblastic tumor is an intermediate-grade tumor, with potential for recurrence and rare metastasis. There are no definite histopathologic, molecular, or cytogenetic features to predict malignant transformation, recurrence, or metastasis. METHODS: A 5-year retrospective study of histopathologically diagnosed inflammatory myofibroblastic tumors of various anatomic sites was conducted to correlate anaplastic lymphoma kinase-1 (ALK-1) expression with histological atypia, multicentric origin of tumor, recurrence, and metastasis. Clinical details of all the cases were noted from the clinical work station. Immunohistochemical stains for ALK-1 and other antibodies were performed. Statistical analysis was done using Fisher exact test. RESULTS: A total of 18 cases of inflammatory myofibroblastic tumors were found during the study period, of which 14 were classical. The female-male ratio was 1:1 and the mean age was 23.8 years. Histologically atypical (four cases) and multifocal tumors (three cases, multicentric in origin) were noted. Recurrence was noted in 30% of ALK-1 positive and 37.5% of ALK-1 negative cases, whereas metastasis to the lung, liver, and pelvic bone was noted in the ALK-1 positive group only. CONCLUSIONS: Overall, ALK-1 protein was expressed in 55.6% of inflammatory myofibroblastic tumors. There was no statistically significant correlation between ALK-1 expression, tumor type, recurrence and metastasis. However, ALK-1 immunohistochemistry is a useful diagnostic aid in the appropriate clinical and histomorphologic context.

Synchronous Appearance of Adenocarcinoma and Gastrointestinal Stromal Tumour (GIST) of the Stomach: A Case Report.

Adenocarcinoma is the most common histological type of gastric tumour, accounting for approximately 95% of all gastric carcinomas. Gastrointestinal stromal tumours (GISTs) are rare mesenchymal neoplasms of the digestive tract. Synchronous adenocarcinoma and gastrointestinal stromal tumour (GIST) occurring in the stomach is rare and very few cases have been reported in literature. Synchronous tumours in the stomach are rarely diagnosed preoperatively. A 63-year-old gentleman was diagnosed with a gastric adenocarcinoma on endoscopic biopsy and underwent surgery. Postoperative histopathologic examination revealed 2 synchronous tumours with both adenocarcinoma and GIST. The adenocarcinoma was determined to be the aggressive tumour based on histologic features. GIST was categorized as a very low risk of malignancy, based on its size and mitosis. The patient underwent chemotherapy for adenocarcinoma. He is under follow up and is currently disease free. Careful histopathologic evaluation is required to detect co-existing rare synchronous tumours. Presence of the second tumour may require additional procedures or protocols.

Papillary Cystadenocarcinoma of the Parotid Gland: A Rare Case Report.

Papillary cystadenocarcinoma is a rare malignant neoplasm of the salivary gland, characterized by noticeable cystic and solid areas with papillary endophytic projections. These tumours lack features that characterize cystic variants of several more common salivary gland carcinomas. It was first described in 1991 by World Health Organization as a separate entity and cystadenocarcinoma with or without papillary component in the AFIP classification. Most of these tumours occurred in the major salivary glands followed by minor salivary glands. Cystadenocarcinoma is the malignant counterpart of cystadenoma. We report a case of papillary cystadenocarcinoma of parotid. A 40-year-old lady presented with gradually progressive swelling below the right ear associated with occasional pain. Clinical and radiological features suggested benign neoplasm. Right lobe superficial parotidectomy was performed. The histopathologic diagnosis showed papillary cystadenocarinoma of the parotid gland. Histologic confirmation of stromal invasion is required to differentiate it from the benign lesion. Conservative wide local surgical excision is the treatment of choice.

Histopathological and Immunohistochemical Evaluation of Meningiomas with Reference to Proliferative Markers p53 and Ki-67.

INTRODUCTION: Meningiomas are slow growing primary central nervous system (CNS) tumours attached to the duramater, which arise from the meningothelial cells of the arachnoid. Grading of meningioma based on histological findings assisted with supplementary immunohistochemical studies, predicts the prognosis of meningioma with good precision. AIM: To evaluate proliferative markers and correlate with various histological subtypes and grade. MATERIALS AND METHODS: A total of 224 meningiomas, diagnosed between January1995 and October 2011were graded according to WHO 2007 criteria. Immunostaining for p53 and Ki-67 markers were performed on 100 cases. RESULTS: There was female predominance. There were 194 Grade I, 24 Grade II and 6 Grade III meningiomas. Brain invasion noted in 18(8%) meningiomas predominantly in grade III followed by grade II. Recurrence was seen in 7 (3.1%) cases, most common in psammomatous followed by angiomatous meningioma. Immunostaining showed p53 positivity in 72.5% of grade I, 83.3% of grade II and all the cases of grade III tumours. Ki-67 Labelling Index (LI) consistently increased from grade I to grade III tumours. CONCLUSION: p53 and Ki-67 LI correlated well with increasing histological grade and biological behaviour of meningioma.

Human epidermal growth factor receptor 2/neu protein expression in meningiomas: An immunohistochemical study.

BACKGROUND: Meningiomas are common slow-growing primary central nervous system tumors that arise from the meningothelial cells of the arachnoid and spinal cord. Human epidermal growth factor receptor 2 (HER2) or HER2/neu (also known as c-erbB2) is a 185-kD transmembrane glycoprotein with tyrosine kinase activity expressed in meningiomas and various other tumors. It can be used in targeted therapy for HER2/neu positive meningiomas. AIM: To correlate the expression of HER2/neu protein in meningiomas with gender, location, histological subtypes, and grade. MATERIALS AND METHODS: It was 3½ years prospective (March 2010-October 2011) and retrospective (May 2008-February 2010) study of histopathologically diagnosed intracranial and intraspinal meningiomas. Clinical details of all the cases were noted from the computerized hospital information system. Immunohistochemistry for HER2/neu protein was performed along with scoring. Statistical analysis was done using Chi-square test to look for any association of HER2/neu with gender, location, grade, and various histological subtypes of meningiomas at 5% level of significance. RESULTS: A total of 100 cases of meningiomas were found during the study period. Of which, 80 were Grade I, 18 were Grade II, and 2 were Grade III meningiomas as per the World Health Organization 2007 criteria. The female-male ratio was 1.9:1 and the mean age was 47.8 years. HER2/neu protein was expressed in 75% of Grade I and 72.2% of Grade II and none of Grade III meningiomas. About 72.7% brain invasive meningiomas showed HER2/neu immunopositivity. CONCLUSION: HER2/neu protein was expressed in 73% of meningiomas. Statistically significant difference of HER2/neu expression was not seen between females and males of Grade I and Grade II/III meningiomas, intracranial and spinal tumors, Grade I and Grade II/III cases, and various histological subtypes of meningiomas.