Lymphoepithelial Carcinoma Originated from the Sinonasal Cavity: Case Report and Literature Review.

Background: Sinonasal lymphoepithelial carcinoma (SNLEC) is a rare neoplasm, representing less than 1% of all types of carcinomas and approximately 3% of head and neck tumors. It can affect the nasopharynx due to the rich lymphoid tissue present in this region. Clinical SNLEC presentation varies, ranging from asymptomatic to nonspecific sinonasal symptoms. We report a case of SNLEC and review the literature for SNLEC presentation, diagnosis, management options, and outcomes. Case Presentation. A 38-year-old male, medically free, presented to the emergency department complaining of nasal obstruction, right facial numbness, persistent right-sided headache, intermittent orbital pain, and a history of on/off epistaxis. Imaging showed a destructive mass in the right sphenoid sinus extending to different sinuses and infratemporal fossa. Biopsy confirmed the diagnosis of SNLEC, with immunohistochemistry being positive for Epstein-Barr virus (EBV) and CK8/18. Induction chemotherapy was started with three cycles of cisplatin and gemcitabine, followed by concurrent chemoradiation therapy. Conclusion: SNLEC is rare, with limited reported cases from around the world. It is mostly seen in adults between their fifth and seventh decades with male predominance. SNLEC is diagnosed using imaging, immunohistochemistry, and EBV testing given its strong association with EBV. Owing to the limited cases, there is no standard approach to treating SNLEC. However, most cases managed with radiation and with and without other modalities showed an excellent response in terms of tumor nonrecurrence.

Pauci-immune Lupus Nephritis in Antiphospholipid Disease: A Diagnostic Challenge.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that leads to immune complex deposition in different organs, especially the kidneys. Pauci-immune lupus nephritis (LN) is a very rare condition. About 40% of SLE cases have a positive antiphospholipid antibody (aPL). So, diagnosing primary antiphospholipid syndrome (APS) in SLE patients is challenging because most symptoms of primary APS can be similar to SLE as described in the American College of Rheumatology/Systemic Lupus Erythematosus International Collaborating Clinics classification criteria for SLE. APS might present as thrombotic microangiopathy (TMA) involving arterioles and glomerular capillaries. We need an adequate renal biopsy to differentiate between lupus and APS nephropathy. A 38-year-old man was diagnosed with biopsy-proven primary APS and LN. Hewas commenced on anticoagulants, pulse steroids, rituximab, and with mycophenolatemofetil as a maintenance therapy in collaboration with the rheumatologist. Here we discuss the occurrence and implications of primary APS and Pauci-immune LN in adults.

Clinicopathologic features and therapy outcome in childhood Hodgkin's lymphoma: a report from tertiary care center in Saudi Arabia.

BACKGROUND: Hodgkin lymphoma (HL) is lymphoid neoplasm usually affecting lymphatic system; it accounts 3.6% of cancers in Saudi Arabia. Modern treatment protocols had shown particular success rates in overall-survival (OS) and event-free-survival (EFS). In our study, we reviewed the medical records of 80 pediatric and young adolescent patients diagnosed HL from January 2006 to July 2020, treated at tertiary care hospital in Riyadh, Saudi Arabia. Demographic, clinical, and pathological data were explored. First line therapy was ABVD, COG, COPP, R-CHOP, or radiotherapy alone in 53/80 (66.4%), 24/80 (30%), 1/80 (1.2%), 1/80 (1.2%), or 1/80 (1.2%) patients; respectively. Response assessment was done by CT + / - PET scan after first 2 cycles then every 2 cycle and end of therapy. Another assessment was done if any clinical suspicion of recurrence. RESULTS: Median age 11 (range 3-16) years. Males to females 1.3:1. Seventy-two out of eighty (90%) patients showed first complete remission (CR1) and maintained remission for median 40 (range 7-136) months. Eight out of eighty (10%) patients showed refractory disease. Nineteen patients received salvage therapy (ICE or ESHAP/brentuximab vedotin or gemcitabine/brentuximab vedotin), 14/19 (73.7%) had 2nd complete remission (CR2) for median time 24 (ranged 9-78) months, while 5/19 (26.3%) did not show any response. Five-year OS and EFS were 95% and 75%. Two patients had 2ry malignant neoplasms, one had AML and died, the other had malignant fibrous histocytoma and still alive. None of our patients had fertility problem. Also, they did not experience chronic pulmonary or cardiotoxicity. Classic Hodgkin's lymphoma: nodular sclerosis subtype was more prominent (55%) than mixed cellularity subtype (22.5%), which is similar to several European and US studies, lymphocyte rich (11.25%) and lymphocyte depleted (0%), while nodular lymphocyte predominant Hodgkin's lymphoma (11.25%). CONCLUSIONS: Our study provided unique descriptive study of childhood HL, in Saudi Arabia, with valuable insight into the long-term outcome and late toxicity. Our results are comparable to other studies in the Middle East and European countries.

Cytological Diagnosis of Plasmablastic Lymphoma Involving the Parotid Gland: A Case Report with Review of the Literature.

Plasmablastic lymphoma (PBL) is a rare subtype of non-Hodgkin's lymphoma that is associated with acquired immunodeficiency syndrome (AIDS), characterized by its association with Epstein-Barr virus (EBV), aggressive nature, and plasmacytic/plasmablastic differentiation. PBL remains a therapeutic and diagnostic challenge. Diagnosis of PBL by fine-needle aspiration cytology (FNAC) is reported infrequently. We herein describe the cytodiagnosis of a rare case of HIV-negative PBL in a 58-year-old man without EBV infection presented by parotid swelling. The current case study highlights the cytomorphologic features that may help to distinguish PBL from other mimics. However, although the cytomorphologic features may suggest PBL, a definitive diagnosis requires additional studies including tissue biopsy and immunohistochemistry, in addition to biochemical investigations and radiological workup to establish the diagnosis and exclude similar conditions. In conclusion, FNAC is a very useful, simple, rapid and reliable procedure for diagnosis of the lymphoma. FNAC provided the earliest clue to diagnosis of PBL, which was later confirmed by tissue biopsy.

Risk Stratification of Thyroid Nodules with Bethesda III Category: The Experience of a Territorial Healthcare Hospital.

Background The Bethesda System for Reporting Thyroid Cytolopathology (TBSRTC) is the standardized category-based reporting system for thyroid nodule (TN) aspirations; however, atypia of undetermined significance/follicular lesion of undetermined significance (Bethesda category III, AUS/FLUS) is the most controversial category. The aim of this study was to identify the degree of malignancy risk and the related risk factors in the surgical pathology of the Bethesda Category III thyroid nodules.  Methods A total of 4074 patients (15-90 years, 81.5% of females) were subjected to retrospective analysis, and a total of 463 nodules were classified as Bethesda Class III and included in the analysis. Once all the thyroid cytopathological slides and ultrasound (US) reports were reviewed, they were classified according to the Bethesda System for Reporting Thyroid Cytology, the American College of Radiology (ACR) and the Thyroid Imaging Reporting and Data System (TI-RADS). Results Among the 463 Bethesda class III nodules, 167 nodules were surgically excised, showing an overall malignancy of 27.6% (n = 46/167). Patients having thyroid-stimulating hormone (TSH) levels of >4.5 mIU/L (35%), TN <2 cm (34.6%), solid or nearly solid (28.7%), highly hypoechoic (58.3%), longer than wide (50%), lobulated (45.5%), punctate echogenic (48.6%), ACR TI-RAD 5 (55.2%) and falling under the ATA category of high suspicion (50%), displayed a higher risk of malignancy (ROM). The chi-square test revealed a strong association between the echogenicity, echogenic foci, ACR TI-RAD and American Thyroid Association (ATA) category between the malignant and benign nodules. The papillary thyroid carcinoma (PTC) follicular variant (39%) and PTC classical (27%) were identified, in this study population, as the commonest forms of thyroid cancer. Conclusion The nodules with AUS/FLUS cytology malignancy rate are comparable with the earlier estimations of other countries. The ACR TI-RAD displayed more accurate diagnostic performances in predicting malignancy in the Bethesda III nodules. However, to confirm the accuracy of the molecular marker tests in specific cytological scenarios, more extensive studies are required in the future.

Generalized lymphadenopathy secondary to isolated extramedullary hematopoiesis as an initial manifestation of primary myelofibrosis.

Extramedullary hematopoiesis (EMH) is a presence of hematopoietic activity in the extramedullary sites. EMH can occur in both benign and malignant hematologic diseases. The liver and spleen are the most common sites, but may also occur infrequently at other sites. EMH often occurs in more than one site and quite rare in an isolated organ. In this study we describe an unusual case of generalized lymphadenopathy secondary to isolated extramedullary hematopoiesis as an initial manifestation of primary myelofibrosis. Computed tomography revealed generalized lymphadenopathy including mediastinal, abdominal and pelvic lymph nodes with extensive illdefined sclerotic lesions throughout the skeletal system suggestive of lymphoma/leukemia. Lymph node biopsy showed no evidence of malignancy or granuloma, however, large abnormal cells with multilobated nuclei were seen scattered in the lymph nodes. These abnormal cells were proved to be megakaryocytes. Granulocytic precursors were less obvious on the H&E section. The diagnosis was determined as EMH in the lymph node. Bone marrow (BM) examination showed hypercellular marrow for patient's age with granulocytic and megakaryocytic proliferation with increase in BM fibrosis and reported as a myeloproliferative neoplasm, consistent with primary myelofibrosis. In summary, although EMH is not always a malignant process; it is important to stress that the patient should be investigated for underlying hematological disorders, when it is noted elsewhere.

Patterns of glomerulonephritis with crescents: Experience at a tertiary medical center in Saudi Arabia.

A series of 78 cases of glomerulonephritis (GN), in which renal biopsy revealed changes of GN associated with crescent formation, were reviewed. Renal pathology findings were correlated with clinical features including patient's age, renal function, and serologic findings. In most of the cases (71.8%), the crescents were due to immune complex-mediated GN. This was followed by pauci-immune GN (20.5%) and anti-glomerular basement membrane antibody (GBM) GN (7.7%). The percentage of glomeruli with crescents was the highest in cases of anti-GBM disease (mean of 93.3%), followed by pauci-immune GBM (mean of 48.2%) and immune complex GN (30.9%). In cases with the pauci- immune GN, there were additional features of glomerular injury including fibrinoid necrosis, disruption of the GBM, and rupture of Bowman's capsule. These changes were generally more pronounced in a subset of pauci-immune GN associated with serum elevation of antineutrophil cytoplasmic antibody (c-ANCA). In biopsies from patient with immune complex disease, systemic lupus erythematosus was the most common cause of crescentic GN.

Fetus-in-fetu. Imaging and pathology.

Fetus-in-fetu (FIF), also known as endoparasitic twin, is a form of asymmetric fetal duplication in which the abnormal developing embryo parasitizes the normal co-twin by attaching internally. Here, we report a case of FIF presented as an intra-abdominal cystic mass, which was first detected during an antenatal ultrasound examination of a 32-year-old Saudi mother. At 34 weeks and 4 days of gestation, she had spontaneous labor and delivered a baby boy. The x-ray and CT examination of the baby boy clearly suggested the presence of limbs, vertebral column, and anomalous blood supply to the mass. After laparotomy and surgical removal, the intra-abdominal mass was subjected to detailed pathological examination. Microscopic studies further showed the presence of brain tissue, gut-like structures, fingers, and limbs. The mass was finally confirmed as FIF.