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Case 1A 67-year-old Asian female was diagnosed with locally advanced high-grade salivary duct carcinoma in June 2011. Molecular analysis revealed human epidermal growth factor receptor 2 (HER-2) amplification. She received adjuvant therapy with carboplatin/paclitaxel/ trastuzumab and maintenance of trastuzumab. Upon disease progression, trastuzumab could not be continued due to lack of financial coverage. Instead, she was treated with compassionate use of lapatinib from April 2013 and standard 5-fluorouracil. Her disease ultimately progressed and she expired later in 2013.Case 2A 68-year-old Asian male was diagnosed with extramammary Paget's disease of the scrotum with HER-2 amplification in May 2011. He received 6 cycles of adjuvant trastuzumab/docetaxel/carboplatin followed by maintenance trastuzumab, which was changed to compassionate use of lapatinib as his insurance did not cover further administration of trastuzumab. He showed clinical benefits from single-agent lapatinib and a combination of lapatinib/capecitabine upon progression to the single-agent lapatinib. Ultimately, he was started on ado-trastuzumab emtansine, which was approved at that time by the FDA for HER-2-positive breast cancer progressed on trastuzumab. He is having clinical and radiographic complete response based on current imaging and normalization of his tumor markers.ConclusionHER-2-targeted therapy should be considered for tumors with HER-2 amplification. In our case series, we would like to emphasize this approach in other rare histologies. Specifically, our patient with extramammary Paget's disease of the scrotum represents the first reported case of a non-breast, non-gastric tumor with HER-2 overexpression with complete clinical and radiographic response to HER-2-targeted therapy.

Simple Summary: Although the high mutation burden in small-cell lung cancer (SCLC) suggests that it should be responsive to immune checkpoint blockade (ICB), relatively few patients have shown durable clinical benefit (DCB). To identify biomarkers of responses, biopsies from 35 patients treated with ICB (21 first-line and 14 second-line) were subjected to transcriptomic analysis and gene signatures were studied. Patients with DCB were compared to those with no clinical benefit (NCB). The response to ICB in the second-line, but not the first-line, setting was associated with gene signatures of inflammation, antigen presentation, interferon responses, and increased CD8 T cells. Our data suggest that responses to ICB in the second-line setting can be predicted by the baseline inflammatory state of the tumor; however, this strong association with inflammation was not observed in the first-line setting, likely because chemotherapy alters the immune milieu allowing a response to ICB. Although immune checkpoint blockade (ICB) is currently approved for the treatment of extensive-stage small-cell lung cancer (SCLC) in combination with chemotherapy, relatively few patients have demonstrated durable clinical benefit (DCB) to these therapies. Biomarkers predicting responses are needed. Biopsies from 35 SCLC patients treated with ICB were subjected to transcriptomic analysis; gene signatures were assessed for associations with responses. Twenty-one patients were treated with ICB in the first-line setting in combination with platinum-based chemotherapy; fourteen patients were treated in the second-line setting with ICB alone. DCB after ICB in SCLC in the second-line setting (3 of 14 patients) was associated with statistically higher transcriptomic levels of genes associated with inflammation (p = 0.003), antigen presentation machinery (p = 0.03), interferon responses (p < 0.05), and increased CD8 T cells (p = 0.02). In contrast, these gene signatures were not significantly different in the first-line setting. Our data suggest that responses to ICB in SCLC in the second-line setting can be predicted by the baseline inflammatory state of the tumor; however, this strong association with inflammation was not seen in the first-line setting. We postulate that chemotherapy alters the immune milieu allowing a response to ICB. Other biomarkers will be needed to predict responses in first-line therapy patients. [ABSTRACT FROM AUTHOR]

Arthroplasty-related pseudotumors are nonneoplastic and noninfectious inflammatory masses that are typically associated with adverse reaction to metal debris. Pseudotumors most commonly occur in the setting of metal-on-metal joint replacements at the hip. However, the presentation of pseudotumor at the shoulder is exceedingly rare. In this article, we reported a case of arthroplasty-related pseudotumor of the scapula. Clinical history, radiologic signs, and tissue analysis are described. Knowledge of this rare diagnosis will support clinical decision making for teams of radiologists, pathologists, oncologists, and orthopaedic surgeons who provide care for patients presenting with suspicious shoulder masses. [ABSTRACT FROM AUTHOR]

This article discusses a case of perinephric myxoid pseudotumor of fat co-occurring with polycystic kidney disease. The patient, a 64-year-old man with end-stage renal disease, had a retroperitoneal mass that was initially suspected to be a well-differentiated liposarcoma. However, further examination revealed that it was a perinephric myxoid pseudotumor of fat. This case is the first known instance of this entity occurring in association with polycystic kidney disease. It is important to recognize this rare condition to avoid misdiagnosis and unnecessary treatment. [Extracted from the article]

Introduction: The rise in primary surgical management of oropharyngeal squamous cell carcinoma has led to varying interpretations of the histopathologic evaluation following a radical tonsillectomy. The oncologic margin may be significantly influenced by the morphologic relations and anatomic dimensions of the palatine tonsil and superior pharyngeal constrictor (SPC) muscle.Objective: The aim of this study was to characterize the gross and histologic anatomic features of the palatine tonsil and SPC muscle following an en bloc radical tonsillectomy.Methods: Radical tonsillectomy specimens were collected from cadaveric and oncologic subjects. Specimens were processed using standard histopathologic techniques and were analyzed by a board-certified head and neck pathologist. The thickness of the SPC muscle and relationship to the tonsillar carcinoma were assessed.Results: Six cadaveric and 10 oncologic specimens were analyzed. The mean minimum SPC width for all cadaveric specimens was 1.02 ± 0.50 mm. The mean minimum width for oncologic specimens was 0.76 ± 0.46 mm. The mean distance from tonsil carcinoma to the lateral specimen margin was 1.79 ± 1.39 mm.Conclusion: Due to the limited width of the SPC muscle, a margin in excess of 2 mm may not be attainable in a transoral radical tonsillectomy. Margin status may be ideally determined by the integrity of the SPC muscle in future oncologic studies, rather than an adequate distance measurement. [ABSTRACT FROM AUTHOR]

Intraductal oncocytic papillary neoplasms (IOPNs) are rare cystic neoplasms composed of oncocytic cells in a complex papillary arrangement. (1) They were first described in the pancreas in 1996 by Adsay [...], Intraductal oncocytic papillary neoplasms (IOPNs) are cystic neoplasms with intraductal growth and complex papillae composed of oncocytic cells. IOPNs have been reported both in the pancreas and biliary tree, and are most likely closely related in these 2 locations. In the pancreas, these rare tumors are now considered 1 of the 4 histologic subtypes of intraductal papillary mucinous neoplasm (IPMN). Significant differences in histology, immunophenotype, and molecular genetics have been reported between IOPNs and other IPMN subtypes. However, there are limited data regarding the clinical behavior and prognosis of IOPNs in comparison to other subtypes of IPMN. We review features of pancreatic IOPNs and discuss the differential diagnosis of other intraductal lesions in the pancreas. (Arch Pathol Lab Med. 2016; 140:992-996; doi: 10.5858/arpa.2014-0595-RS)