IMPORTANCETherapies for patients with advanced well-differentiated neuroendocrine tumors (NETs) have expanded but remain inadequate, with patients dying of disease despite recent advances in NET therapy. While patients with other cancers have seen long-term disease control and tumor regression with the application of immunotherapies, initial prospective studies of single-agent programmed cell death 1 inhibitors in NET have been disappointing.OBJECTIVE To evaluate the response rate following treatment with the combination of the vascular endothelial growth factor inhibitor bevacizumab with the programmed cell death 1 ligand 1 inhibitor atezolizumab in patients with advanced NETs. DESIGN, SETTING, AND PARTICIPANTS This single-arm, open-label nonrandomized clinical study in patients with rare cancers included 40 patients with advanced, progressive grade 1 to 2 NETs (20 with pancreatic NETs [pNETs] and 20 with extrapancreatic NETs [epNETs]) treated at a tertiary care referral cancer center between March 31, 2017, and February 19, 2019. Data were analyzed from June to September 2021. INTERVENTIONS Patients received intravenous bevacizumab and atezolizumab at standard doses every 3 weeks until progression, death, or withdrawal. MAIN OUTCOMES AND MEASURESThe primary end point was objective radiographic response using Response Evaluation Criteria in Solid Tumors, version 1.1, with progression-free survival (PFS) as a key secondary end point. RESULTSFollowing treatment of the 40 study patients with bevacizumab and atezolizumab, objective response was observed in 4 patients with pNETs (20%; 95% CI, 5.7%-43.7%) and 3 patients with epNETs (15%; 95% CI, 3.2%-37.9%). The PFS was 14.9 (95% CI, 4.4-32.0) months and 14.2 (95% CI, 10.2-19.6) months in these cohorts, respectively. CONCLUSIONS AND RELEVANCEIn this nonrandomized clinical trial, findings suggest that clinical responses in patients with NET may follow treatment with the combination of bevacizumab and atezolizumab, with a PFS consistent with effective therapies.

The calcifying epithelial odontogenic tumor (CEOT), also known as the Pindborg tumor, is a benign locally invasive neoplasm. Common variants of CEOT include noncalcifying, Langerhans cell, bone and cementum forming and clear cell, which have a prognostic significance. Pigmented variants are known to occur in other odontogenic tumors. However, a definitive pigmented variant of CEOT has not been reported in literature so far. Here, we report the first case of pigmented Pindborg tumor arising from the maxilla in a young female. The pigment was demonstrated as melanin by staining and confirmed by immunohistochemistry. The pigmented variant of CEOT did not recur within 18 months postsurgery. Our report indicates that it is essential to recognize the pigmented variant. We discuss the common variants of CEOT and potential histogenesis of the pigmented variant. Further studies are required to reveal the histogenesis of melanocytes and their pathological significance in the odontogenic tumors.

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Osteoclast-like giant cell tumor of the pancreas is a rare non-endocrine neoplasm composed of reactive multinucleated giant cells admixed with mononuclear stromal cells. We report a case of osteoclast-like giant cell tumor of the pancreas in a 58-year-old female with vague clinical symptoms. Endoscopic ultrasound-guided aspirate from the mass revealed numerous characteristic osteoclast-like giant cells.

Mixed- phenotype acute leukaemia (MPAL) is very rare and accounts for less than 4% of acute leukaemia. Most cases of MPAL described in literature, are of T/myeloid or B/myeloid phenotype. MPAL T/B cell lineage is exceptional and occasional cases reported so far, are leukaemia with bone marrow involvement. Our case, on immunophenotyping, exhibited evidence of T and B- Lymphoid lineage. It could be diagnosed neither as MPAL, because the bone marrow was not involved, nor as lymphoblastic lymphoma because of the bi phenotypic expression of both T and B cell antigens. Hence, we reported it as Mixed phenotypic (T cell/B cell) Lymphoblastic Lymphoma. This is the first case, extra medullary as well as extra lymphoid in location, presenting as right elbow synovial lesion. We also discuss the potential diagnostic pitfalls and emphasise the importance of Immunohistochemistry in diagnosis of lymphoblastic lymphomas.

Materials and Methods After obtaining the institutional ethics committee, this retrospective study on 28 foetal autopsies performed in the department of Pathology, Saveetha Medical College, over a period of three years from November 2012 to November 2015 was performed. The cases, for which all the autopsy data were available, were included in the study. Macerated and autolyzed foetuses were excluded from the study. All the details in autopsy reports including gestational age, sex of the foetus, external examination findings, anthropometric measurements, internal examination findings, findings of the examination of head & neck, brain and spinal cord, results of examination of placenta and umbilical cord were retrieved from the departmental achieves and analysed. Cunningham & Hollier classification [5] was used to divide cases into foetal, placental or maternal categories as: Foetal (25-40%)-chromosomal anomalies, non-chromosomal birth defects, non-immune hydrops, infections; Placental (25-35%)-abruption, foetal-maternal haemorrhage, cord accident, placental insufficiency, intra partum asphyxia, placenta previa, twin to twin transfusion, chorioamnionitis; Maternal (5-10%)-diabetes, hypertensive disorders, trauma, abnormal labour, sepsis, uterine rupture, postterm pregnancy, drugs, antiphospholipid antibodies, unexplained.

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Previously ectopic breast tissue was thought to be derived from the caudal remnants of the primitive embryonic milk ridges; anogenital mammary-like glands are presently considered as normal constituents of the anogenital region. We report a case of young female, who presented with an anal papilloma. Histopathological examination revealed extensive fibrocystic changes in anogenital mammary-like glands. To date, a lot of benign changes and a wide range of benign and malignant neoplasms have been reported in these glands. However, extensive fibrocystic change of these glands in anal region is very rare. In addition, fibrocystic disease of anal mammary glands, masquerading clinically as an anal papilloma, has not been reported in literature. Hence, it is essential for clinicians and the pathologists to be aware of such a rare presentation. The features of fibrocystic disease in perianal region are also discussed.