Role of CD34-immunopositive Microvascular Density in Juvenile Nasopharyngeal Angiofibroma.

Objective: To assess the microvascular density (MVD) in juvenile nasopharyngeal angiofibroma (JNA) with CD34 immunostaining and evaluate its relationship with clinico-demographic features. Methods: This prospective study included patients with JNA undergoing endoscopic excision. The histopathological specimen was stained using CD-34 antibodies to calculate MVD. MVD and clinico-demographic features were correlated. Results: The study included 12 patients with a median age of 15.5 years. The mean MVD was 39 vessels/high power field (range 5 to 151 vessels). MVD was significantly associated only with the volume of tumour (r = 0.65, p = 0.02). The recurrence occurred in one patient with an MVD of 107. The median follow-up was 38 months. Conclusion: MVD is significantly associated with tumour volume in JNA, which implies a robust role of angiogenesis in the pathology of the tumour. Also, higher MVD may be a risk factor for recurrence.

Dermoscopy of hypopigmented macules unveiling genetic diagnosis of tuberous sclerosis complex type 2 in an infant presenting with sacral chordoma.

A 2-month-old male with surgically resected sacral chordoma presented with multiple hypopigmented macules showing characteristic patchy, sharply demarcated areas of pigment network on dermoscopy. These dermoscopic findings were suggestive of the ash-leaf macules of tuberous sclerosis over other common hypopigmented macules in neonates. Chordomas presenting in early childhood in the sacral location have been reported as a rare manifestation of tuberous sclerosis complex. The combination of these findings led to a diagnosis of tuberous sclerosis, confirmed with the finding of a heterozygous TSC2 gene deletion; treatment with sirolimus resulted in regression of cardiac rhabdomyomas and hypopigmented macules.

Assessment of MUC5AC and MUC2 Immunoexpression in Glandular Odontogenic Cysts, Dentigerous Cysts, and Mucoepidermoid Carcinomas.

Glandular odontogenic cysts (GOCs) and dentigerous cysts may show mucous metaplasia. Central mucoepidermoid carcinoma is very rare and mostly associated with dental cysts. It is hypothesized that odontogenic cysts showing mucus differentiation in their lining, have a propensity to transform into MEC. The present study is the first attempt to explore the relationship between odontogenic cysts [GOCs and dentigerous cysts with mucus metaplasia (DCMM)] and MEC by evaluating immunoexpression of MUC5AC and MUC2. Immunoexpression of MUC5AC and MUC2 was evaluated semiquantitatively in GOCs (20 cases), DCMMs (20 cases), and MECs (20 cases). The percentage of positive cells, intensity, and localization of immunoexpression were assessed for each marker in all cases. Of GOCs, DCMMs, and MECs cases, 85%, 70%, and 80%, respectively, were immunopositive for MUC5AC. Strong cytoplasmic immunoreactivity for MUC5AC was noted, particularly in mucous cells present diffusely within MECs. However, the immunoreactivity was limited to the epithelial lining of GOCs and DCMMs. Most of the MECs (60%) showed more than 25% positivity for MUC5AC, followed by GOCs, and the least in DMMCs. Mild cytoplasmic and nuclear positivity of MUC2 was noted only in epithelial lining cells of 70% GOCs and 45% DCMMs. Whereas, 55% of MECs displayed moderate to strong cytoplasmic and membranous immunopositivity for MUC2 exclusively within mucous cells. As MECs showed strong MUC5AC immunoreactivity in mucous cells, immunoexpression of MUC5AC in odontogenic cysts with mucus cells can possibly explain the pathogenesis of MEC from cysts. However, the variable expression of MUC2 did not give any strong evidence regarding its role as a marker.

Clinico-pathological Factors in Malignant Transformation of RRP.

Various clinico-pathological factors play role in the papilloma proliferation and pathogenesis of Recurrent respiratory papillomatosis (RRP). However, it is not known if they are directly responsible for malignant transformation of these papillomas or not. We did this study to elucidate any such association. The most recent debrided tissue of RRP in 20 patients was evaluated for p16 expression, VEGF estimation (tissue expression and serum levels), and tissue HPV DNA concentration. The final histopathology results were then correlated with these pathological factors and with clinical factors like duration of illness, age of onset of symptoms, extent of disease, etc. Squamous papilloma was seen in 60%, dysplasia in 25%, and squamous cell carcinoma (SCC) in 15% of the patients. Positive immunostaining for p16 (staining in ≥70% of tumor cells) was seen only in one case, which was SCC. There was no statistically significant difference between p16 expression, tissue VEGF expression, serum VEGF levels, and tissue HPV DNA in any of the histological groups. The mean age of disease onset was significantly higher in patients with SCC (p = 0.03). A significantly higher number of patients with dysplasia had tracheobronchial involvement (p = 0.022). We concluded that no single pathological factor is solely responsible for development of malignancy in RRP, whereas clinical factors like tracheobronchial involvement and age of onset may contribute to development of dysplasia or carcinoma.

Trichilemmal carcinoma metastatic to parotid gland: First report of fine needle aspiration cytology features.

Trichilemmal carcinoma is a rare malignant cutaneous adnexal neoplasm arising from the outer root sheath of the hair follicle. Majority of cases occur on sun-exposed sites such as the face, scalp and neck, making them easily amenable to being biopsied and subjected to histological examination for definitive diagnosis. Thus, cytological features of trichilemmal carcinoma have not been described till date. Trichilemmal carcinoma is a low-grade malignancy, albeit with potential to metastasize to regional lymph nodes and distant sites. We report the case of trichilemmal carcinoma of scalp that metastasized to cervical lymph nodes and parotid gland and underwent fine-needle aspiration cytology (FNAC) from the parotid lesion. The aspirate showed tightly cohesive cell clusters with sharp borders. Tumour cells ranged from basaloid with scant cytoplasm to those with abundant cytoplasm. Nuclei were vesicular, with inconspicuous to prominent nuclei. Intercellular bridges, masses of keratin, and fragments of desmoplastic stroma were present, closely recapitulating histological features of trichilemmal carcinoma, which enabled diagnosis as metastasis. Cell block showed similar tumour fragments with evidence of differentiation towards outer root sheath. FNAC is the first-line investigation to obtain a tissue diagnosis of masses in the head and neck region. Although rarely encountered, the lack of knowledge of cytological features of trichilemmal carcinoma may hamper its FNAC diagnosis at metastatic sites. When intraparotid metastases occur, they may be mistaken as primary salivary gland carcinoma. Thus, awareness of the cytological features of this tumour must be raised among cytopathologists to enable accurate diagnosis and further management.

Myeloblastic meningeal neoplasm: an unusual natural history.

Myeloid sarcoma is a very rare extramedullary malignant tumour, most often associated with acute myeloid leukaemia. We report the case of a man in his early 20s who presented with chronic headache, raised intracranial pressure and progressive vision loss of 2 years duration with no systemic manifestations. He had a history of myeloid sarcoma of the left thigh 15 years ago, treated with external beam radiotherapy and in complete remission for more than 13 years. However, the progressive blindness remained unexplained for 2 years, and he was eventually diagnosed with isolated meningeal relapse without marrow or systemic involvement. Imaging revealed subarachnoid haemorrhage, diffuse leptomeningeal enhancement and involvement of lower dorsal cord and conus, and cerebrospinal fluid cytology showed myeloid blasts. He was managed with intrathecal chemotherapy and craniospinal irradiation, after which he had mild improvement in vision.

Liver Metastasis from Nasopharyngeal Carcinoma Diagnosed on Fine Needle Aspiration Cytology.

A 12-year-old boy presented with abdominal distention for 1 year. On examination, he had massive hepatomegaly. Facial swelling in the maxillary region, palpable left cervical lymph nodes, and a nasal twang to his voice were detected. Imaging showed multiple hypodense liver lesions, necrotic mediastinal and hilar lymph nodes, multiple lytic-sclerotic skeletal lesions, and lesions in the nasopharynx and maxilla. Fine needle aspirate (FNA) from the cervical lymph node yielded blood. FNA from the liver showed singly dispersed and cohesive clusters of tumor cells, with interspersed neutrophils and tingible body macrophages. Cells had scant to moderate fragile cytoplasm, enlarged vesicular nuclei, and prominent nucleoli. Immunohistochemistry on cell block revealed positivity for cytokeratin and Epstein-Barr virus (EBV)-Latent Membrane Protein-1 (LMP1). A diagnosis of metastatic nasopharyngeal carcinoma was made, and was confirmed on a subsequent biopsy from the femur.

Relevance of Partial p16 Immunostaining in Oral Squamous Cell Carcinoma and Co- Relation with HPV DNA Status.

INTRODUCTION: Immunostaining criteria for p16 positivity in oropharyngeal squamous cell carcinoma have been laid down by College of American Pathologists (CAP) and the American Society of Clinical Oncology (ASCO). The staining should be of moderate to strong intensity seen in 70 percent of the tumor cells. Recent studies have pointed out that a small minority of cases are missed using p16 as the surrogate marker at above mentioned cut off. By convention the same criteria have been used for oral squamous cell carcinoma. MATERIAL AND METHODS: The authors revisited the results of their previous study where immunohistochemistry for p16 was found to be positive by AJCC criteria in 139 out of 800 cases of oral squamous cell carcinoma. For this study, all the p16 immunonegative cases (by AJCC criteria) were analysed again for partial staining patterns, defined for this study as cases with 50-75% cells showing 2+/3+ intensity of nuclear p16 immunostaining and for basal predominant pattern of immunostaining. These cases were subjected to HPV DNA PCR. RESULTS: Out of the 661/800 cases found to be negative for p16 immunohistochemistry, a total of 34/800(4.25%) showed partial staining based on the criterion of 50-75% cells showing p16 immunostaining intensity of 2/3+.The basal predominant pattern of immunostaining for p16 was seen in 43/800 (5.38%) cases. When these cases were subjected to HPV DNA analysis, 11/34 (32.35%) of the cases showing partial staining and 02/43 (4.7%) of the cases showing basal predominant pattern of p16 immunostaining were found to be HPV-DNA positive. CONCLUSION: The inclusion of partial immunostaining patterns of p16 in HPV analysis of oral squamous cell carcinoma can improve our understanding of HPV driven oral squamous cell carcinoma.

Diagnosis of Adenoid Cystic Carcinoma with Striking Tubular Hypereosinophilia by MYB and EWSR1 Breakapart Fluorescence In Situ Hybridization.

BACKGROUND: Adenoid cystic carcinoma (AdCC), associated with MYB/MYBL1 gene rearrangements, shows epithelial and basaloid myoepithelial cells arranged in tubular, cribriform and solid patterns. Variations from this classic morphology make diagnosis challenging, necessitating molecular testing. AdCC with striking tubular hypereosinophilia (AdCC-STE) is one such recently described histological subtype. METHODS: A 52-year-old female presented with a floor of mouth swelling for two months, diagnosed elsewhere as polymorphous adenocarcinoma (PAC). A biopsy was obtained. With a diagnosis of oncocytic neoplasm, wide excision of the tumor was undertaken. Histological examination, fluorescence in situ hybridization (FISH) and ultrastructural examination were performed. Archival cases of PAC and epithelial myoepithelial carcinoma (EMC) were reviewed, and MYB immunostaining and FISH were performed to identify potential AdCC-STE cases. RESULTS: The excised tumor from the index patient showed bilayered tubules, micropapillae and cribriform pattern. Luminal cells with hypereosinophilic to clear cytoplasm were surrounded by flattened abluminal cells. Focally, basophilic matrix was seen within sharply demarcated pseudocystic spaces. FISH revealed MYB and EWSR1 gene rearrangements, confirmatory of AdCC-STE. Electron microscopy showed features consistent with AdCC; however, mitochondria were not prominent. Among 14 archival PACs, two showed MYB immunopositivity; one showed MYB rearrangement but was classical AdCC. Among 35 EMC, one case showed MYB immunoreactivity and eosinophilia of luminal cells but lacked MYB/MYBL1 rearrangement. CONCLUSION: Awareness of unusual histological subtypes of AdCC, such as AdCC-STE, is imperative, as it may be misdiagnosed as PAC and EMC, among others. Presence of basophilic matrix and squamoid morules in a biphasic tumor even with hypereosinophilic rather than basaloid myoepithelial appearance should raise suspicion for AdCC-STE, and prompt molecular testing for confirmation. With wider accessibility, lower cost and significantly shorter turn-around-time when compared to RNA sequencing, FISH can be employed for confirmation of diagnosis, especially in low- and middle-income countries.

Role of immunohistochemistry in diagnosing round-cell tumours affecting the oral and maxillofacial regions.

Background: One of the most challenging spectra of lesions in the oral and maxillofacial region (OMFR) are round-cell tumours (RCTs). They show a considerable degree of overlap in microscopy and immunophenotypes. The main aim of this study is to analyse the spectrum of RCTs encountered in the oral and maxillofacial regions. We emphasise the role of immunohistochemistry (IHC) which in conjunction with histological, clinical, and imaging findings is necessary for their correct characterisation. The secondary objectives are to discuss differential diagnosis, workflow, and diagnostic algorithm for round-cell lesions affecting the OMFR. Methods: Formalin-fixed, paraffin-embedded sections of RCTs were retrieved from the archives of the Department of Oral Pathology (January 2018 to March 2020). These cases were analysed by three pathologists independently by evaluating haematoxylin and eosin-stained sections, and immunohistochemical markers employed to characterise these lesions. Results: Under the spectrum of RCTs, 11 cases (0.53%) were diagnosed with a predominance of non-Hodgkin lymphoma (55%) followed by Ewing sarcoma (18%). The remaining were Langerhans cell histiocytosis (9%), neuroendocrine carcinoma (9%), and sinonasal undifferentiated carcinoma (9%). Except for one case, in all cases, the final diagnosis was established with the use of adjunctive IHC. Conclusion: RCTs can pose a diagnostic challenge for inexperienced oral pathologists. Thorough knowledge of the differentials of RCT occurring in oral and maxillofacial is helpful. An algorithm-based diagnostic approach incorporating the clinical, imaging, and histomorphological findings and immunohistochemical evaluation can help in minimizing diagnostic confusion and errors.

Comparison of 68Ga-PSMA-HBED-CC and 18F-FDG PET/CT in the Evaluation of Adenoid Cystic Carcinoma-A Prospective Study.

PURPOSE OF STUDY: 18F-FDG PET/CT plays a major role in diagnosis and staging of head and neck cancer; however, FDG has lower uptake in adenoid cystic carcinoma (AdCC). Prostate-specific membrane antigen (PSMA) expression is found to be associated with endothelial cells or tumor neovasculature in malignant AdCC and salivary duct carcinoma. Thus, present study is aimed to compare the role of 68Ga-PSMA and 18F-FDG PET/CT in patients with primary and/or metastatic AdCC. MATERIALS AND METHODS: Histopathologically proven AdCC patients were intravenously injected with 370 MBq (10 mCi) of 18F-FDG and 111-185 MBq (3-5 mCi) of 68Ga-PSMA. Images were acquired at 60 and 45 minutes postinjection for 18F-FDG and 68Ga-PSMA, respectively, on dedicated PET/CT scanners. Visual and semiquantitative analyses of PSMA expression in regional and metastatic sites were performed by 2 experienced nuclear medicine physicians. RESULTS: Seventeen patients (7 men, 10 women) having mean age of 44 ± 14.19 years were prospectively included in the study. Of 17 patients, FDG PET/CT was performed in only 14 (82%) patients. PSMA and FDG uptakes were seen at the primary site in 16 (94%) and 13 (93%) patients, respectively, whereas 1 patient was postradical tumor excision. Lung lesions (n = 7) and lymph nodes (n = 5) were detected on both FDG and PSMA PET scans. However, cerebellar and meningeal metastasis (n = 1, 6%) and bony lesions (n = 2, 12%) were detected only on PSMA PET/CT but not visualized on FDG PET/CT scan. CONCLUSIONS: PSMA may have theranostic importance in unresectable or metastatic AdCC, besides having a role in staging/restaging.

HPV-Associated Squamous Cell Carcinoma of the Eyelid: Diagnostic Utility of p16 Immunohistochemistry and mRNA In Situ Hybridization.

BACKGROUND: High-risk (HR) Human papillomavirus (HPV) has been implicated in pathogenesis of squamous cell carcinomas (SCC) at several sites with mucocutaneous junctions, including the head and neck. SCC is the second most common eyelid malignancy. However, its association with transcriptionally active HR-HPV has not been adequately studied. METHODS: Two index cases of eyelid HPV-associated SCC are described in detail. A retrospective cohort of eyelid SCC was examined for p16 immunoexpression. Cases demonstrating p16 positivity or equivocal staining were subjected to high-risk HPV mRNA in situ hybridization (ISH). Quantitative real-time PCR (qPCR) was performed in mRNA ISH-positive cases for HPV genotyping. RESULTS: The two index patients were older adult females, with upper eyelid tumours. On histology, both tumours were non-keratinizing SCC with trabecular and nested architecture reminiscent of oropharyngeal HPV-associated non-keratinizing SCC, prompting p16 immunohistochemistry, which was positive. HR-HPV mRNA ISH was positive, and qPCR detected HPV16 in both cases. Three of 20 (15%) archival cases showed p16 immunopositivity and two (10%) showed equivocal staining. However, mRNA ISH was negative. All cases showing p16 immunostaining and lacking HR-HPV were keratinizing SCCs. Thus, 9% of all eyelid SCC examined demonstrated HR-HPV. CONCLUSION: The prevalence of HR-HPV in eyelid SCC is low in Indian patients. HPV-associated SCC may mimic commoner eyelid carcinomas as it lacks overt keratinization. In basaloid-appearing eyelid carcinomas, p16 immunopositivity should be followed by reflex HR-HPV mRNA ISH, as p16 immunohistochemistry alone has low specificity. The prognostic role, if any, of HPV association needs further evaluation.

Tumour-infiltrating lymphocyte subsets and their individual prognostic impact in oral squamous cell carcinoma.

AIMS: Current understanding of oral squamous cell carcinoma (OSCC) is incomplete with regard to prognostic factors that lead to the considerable heterogeneity in treatment response and patient outcomes. We aimed to evaluate the impact of individual tumour-infiltrating lymphocyte (TIL) subsets on prognosis as a possible rationale for this, in a retrospective observational study. METHODS: Immunohistochemistry was performed to quantitatively assess cell densities of CD3+, CD20+, CD4+, CD8+ and FOXP3+TIL subsets in 50 surgically treated OSCC cases. Results were correlated with disease-free survival (DFS) and overall survival (OS). Receiver operating characteristic curve analysis and Youden index were applied to determine prognostically significant cut-off values. RESULTS: Mean counts for CD3+, CD4+, CD8+, CD20+ and FOXP3+TILs were 243, 52, 132, 53 and 116 cells per high power field, respectively. High CD8+ and low FOXP3+TIL counts, and high ratio of CD8:FOXP3 were significantly associated with longer DFS and OS, as well as with improved tumour-host interface parameters. CONCLUSIONS: Host immune response and its interaction with cancer cells have a significant impact on OSCC outcomes, with some TIL subsets being more clinically relevant than others. High cytotoxic T-cell (CD8) and low Treg (FOXP3) counts, and high cytotoxic T-cell to Treg (CD8:FOXP3) ratio are significantly associated with favourable prognosis. These results may serve as a leading point in identifying novel therapeutic agents that can redesign the tumour immune microenvironment by reducing infiltrating FOXP3-lymphocytes, and modifying their signalling pathways.

Can Superoxide Anions Predict the Malignant Potential of Carotid body Tumor? - A Pilot Study.

Currently, there is no consensus on estimating the malignant potential of Carotid Body Tumor (CBT) and the only way to predict a metastatic CBT is through DOTANOC Positron Emission Tomography (PET) scan. There is a well-established correlation between CBT and superoxide anions inside tumor cells. The purpose of this pilot study was to measure superoxide anions inside CBT cells and find if these can be used as marker to predict malignant potential of CBT. The results were also co-related with findings of DOTANOC PET scan retrospectively. The CBT tissue from 10 patients was stained using a fluorogenic dye and superoxide anions were measured by analysis of fluorescent image. The patients were divided into two groups - First group with four patients having potentially malignant CBT based upon clinico-surgical characteristics and second group with the rest of the six patients. It was seen that the superoxide anions were highest in the first group which included patients with metastatic carotid body tumor, patients with multiple paragangliomas and patient with positive family history (p = 0.011). The same patients also had metastasis and multiple tumors detected on DOTANOC PET scan. It was concluded that measuring superoxide anions in excised tumor tissue can be used to estimate malignant potential of CBT and can identify patients who truly require DOTANOC PET scan; without affecting the treatment, as it is an expensive investigation involving ionizing radiation and may not be available in all centres. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-03623-6.

Evaluation of high-risk human papillomavirus in sinonasal papillomas and squamous cell carcinomas.

The sinonasal tract is considered a second hotspot for human papillomavirus (HPV)-related tumors in the head and neck, with HPV being identified in up to 62% of squamous cell carcinomas (SCCs) and 38% of papillomas. There is limited data from geographical regions with low prevalence of high-risk (HR)-HPV on the association of HR-HPV in sinonasal neoplasms and on utility of p16 as a surrogate marker. p16 immunohistochemistry, HR-HPV mRNA ISH and quantitative real-time PCR (qPCR) were performed on a retrospective cohort of sinonasal papillomas and SCCs. KRAS mutation analysis was done in oncocytic papillomas. p16 positivity was present in 22/142 cases (15.5%) including eight inverted papillomas, one oncocytic papilloma (OP), and 13 SCC. Among these, mRNA ISH showed HR-HPV in the OP and two SCC, while another SCC was found to harbour HPV18 by qPCR. Two HPV-associated SCCs had foci of OP. mRNA ISH was negative in all p16 negative cases. p16 immunohistochemistry showed 68% concordance with mRNA ISH, and had sensitivity and negative predictive value of 100%; specificity was 67%, and positive predictive value was 14.3%. Association with HR-HPV in sinonasal papillomas and SCC is rare, and may be seen in cases demonstrating oncocytic morphology. p16 immunohistochemistry has low specificity and positive predictive value in low-prevalence populations; thus, reflex direct HR-HPV testing should be performed in p16 immunopositive cases. This two-step approach is viable in resource-limited settings, as the proportion of p16 positive cases is small.

Virological and cytological changes in tears and conjunctiva of patients with COVID-19.

Purpose: To analyze the presence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in tears/conjunctival epithelium and assess the cytomorphological changes in the conjunctival epithelium of coronavirus disease 2019 (COVID-19) patients. Methods: In this pilot study, patients with moderate to severe COVID-19 were recruited from the COVID ward/intensive care unit of the institute. Tears and conjunctival swabs were collected from COVID-19 patients and sent to the virology laboratory for reverse transcription polymerase chain reaction (RT-PCR) testing. Conjunctival swabs were used to prepare smears, which underwent cytological evaluation and immunocytochemistry for SARS-CoV-2 nucleocapsid protein. Results: Forty-two patients were included. The mean age of participants was 48.61 (range: 5-75) years. Seven (16.6%) patients tested positive for SARS-CoV-2 ribonucleic acid in tears samples, four (9.5%) of which were positive on conjunctival swab by RT-PCR in the first test. Cytomorphological changes were observed significantly more in smears from patients with positive RT-PCR on tear samples, including bi-/multi-nucleation (p = 0.01), chromatin clearing (p = 0.02), and intra-nuclear inclusions (p < 0.001). One case (3.2%) showed immunopositivity for SARS-CoV-2; this patient had severe disease and the lowest Ct values for tear and conjunctival samples among all positive cases. Conclusion: Conjunctival smears from patients with COVID-19 revealed cytomorphological alterations, even in the absence of clinically significant ocular infection. However, viral proteins were demonstrated within epithelial cells only rarely, suggesting that although the conjunctival epithelium may serve as a portal for entry, viral replication is possibly rare or short-lived.

Sclerosing epithelioid fibrosarcoma of the kidney.

A woman in her 20s presented with headache and back pain and was found to have a left renal mass with bony metastases. She underwent nephrectomy, and on histopathology was initially diagnosed with stage 4 clear cell sarcoma of the kidney. She underwent palliative radiation and chemotherapy; however, the disease progressed and she came to our centre. We started her on second-line chemotherapy and submitted her tissue blocks for review. Due to her age and lack of sclerotic stroma in the tissue, we had our doubts about the diagnosis and hence, tissue sample was submitted for next-generation sequencing (NGS). NGS detected an EWSR1::CREBL1 fusion, clinching the final diagnosis of sclerosing epithelioid fibrosarcoma of the kidney, a singular diagnosis rarely reported in the literature. Currently, the patient is post her third line of chemotherapy, is on maintenance, and is doing well and has resumed her daily activities.

Epithelioid inflammatory myofibroblastic sarcoma arising in the maxilla: first reported case in the jaws.

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of inflammatory myofibroblastic tumors located mostly in the intra-abdominal region. Herein, we present a case of a 32-year-old male with a lobulated growth in the right maxilla. Radiology revealed a solitary osteolytic lesion with an irregular margin, causing buccal and palatal cortex erosion. Histopathology revealed a tumor composed of spindle-shaped fascicles that blend into sheets of round to ovoid-shaped epithelioid cells, areas of myxoid changes, and necrosis. Tumor cells showed a moderate amount of eosinophilic cytoplasm, large vesicular nuclei with coarse chromatin, nuclear pleomorphism, and increased mitoses. Tumor cells were immunopositive for ALK-1, focal positivity for smooth muscle actin, panCK, epithelial membrane antigen, and immunonegativity for CD 30, desmin, CD34, and STAT6. P53 showed a wild-type staining pattern, and INI-1 expression was retained. The Ki-67 proliferative index was 22%. To the best of our knowledge, this is the first case of EIMS occurring in the maxilla.

The Role of Serum Galactomannan Assay as a Potential Surrogate Biomarker for Fungal Microinvasion in Allergic Fungal Rhinosinusitis.

We conducted this study to determine if serum galactomannan (GM) can be used as a marker to implicate the invasiveness of allergic fungal rhinosinusitis (AFRS), and correlate this value with the aggressiveness of disease documented via computed tomography (CT). All paranasal CT scans done for AFRS patients prospectively over a five-year period (2015-2019) were included. An indigenous 20-point score was used to document the extent of bone erosion seen on CT, wherein a higher score meant a greater extent of bone erosion. It was then correlated with serum GM scores. The median CT scores of galactomannan-positive (GM+) patients were compared with the median CT scores of galactomannan-negative (GM-) patients 3 using Mann-Whitney U test. The patients were divided into five groups based on the extent of disease-No bone erosion, erosion of only sinus wall/orbit, 3 erosion of orbit and skull base, erosion of only skull base and lateral extension of disease into infratemporal fossa (ITF). Subgroup analysis was conducted over mean GM values in these groups using ANOVA test. p-value < 0.05 was considered significant. Statistical analysis was performed using SPSS version 25.0. A total of 92 patients were included (56 males, 36 females). No statistically significant difference was found (p-value = 0.42) between the CT scores of galactomannan-positive (GM+) group and galactomannan-negative (GM-) group. The mean GM scores amongst the five sub-groups did not show a statistically significant difference. Serum galactomannan values correlate poorly with aggressiveness of disease quantified on non-contrast CT of paranasal sinuses.

Top 10 Clear Cell Head and Neck Lesions to Contemplate.

BACKGROUND: Optically clear cytoplasm may occur in neoplastic and non-neoplastic conditions, either as a characteristic feature of a disease entity or as a morphologic rarity, potentially creating diagnostic dilemmas in various organ systems. In the head and neck, clear cell change can occur in lesions of salivary, odontogenic, thyroid, parathyroid, or sinonasal/skull base origin, as well as in metastases to these regions. METHODS: This review elaborates the top ten clear cell lesions in the head and neck, emphasizing their distinguishing histologic, immunohistochemical, and molecular attributes, and presents a rational approach to arriving at an accurate classification. RESULTS: Cytoplasmic pallor or clearing may be caused by accumulations of glycogen, lipid, mucin, mucopolysaccharides, water, foreign material, hydropic organelles, or immature zymogen granules. Overlapping morphologic features may present a diagnostic challenge to the surgical pathologist. Similarity in immunohistochemical profiles, often due to common cell type, as well as rare non-neoplastic mimics, furthers the diagnostic conundrum. CONCLUSIONS: The top ten lesions reviewed in this article are as follows: (1) clear cell carcinoma (salivary and odontogenic), (2) mucoepidermoid carcinoma, (3) myoepithelial and epithelial-myoepithelial carcinoma, (4) oncocytic salivary gland lesions, (5) squamous cell carcinoma, (6) parathyroid water clear cell adenoma, (7) metastatic renal cell carcinoma (especially in comparison to clear cell thyroid neoplasms), (8) sinonasal renal cell-like adenocarcinoma, (9) chordoma, and (10) rhinoscleroma.