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Cureus 2022 Jun 16;14(6):e26012. Epub 2022 Jun 16.

Hematology and Oncology, East Orange Veterans Affairs Medical Center, East Orange, USA.

Extrapulmonary small cell carcinomas (ESCCs) are poorly differentiated neuroendocrine tumors that are characterized by an aggressive course and poor survival rates. While these tumors can be found anywhere in the body, presentations of lesions in the orbit are exceedingly rare. We present the case of a 47-year-old man who presented with blurry vision, lacrimation, and tenderness of his right eye, as well as a small but palpable temporal mass. Read More

Zhongguo Fei Ai Za Zhi 2022 May 16;25(5):303-310. Epub 2022 May 16.

Department of Pathology, Peking University Cancer Hospital & Institute; Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Beijing 100142, China.

Background: The expression of programmed cell death ligand 1 (PD-L1) as a biomarker for immunotherapy in non-small cell lung cancer (NSCLC) is routinely detected in clinical pathology department. However, the spatial heterogeneity of PD-L1 expression in intrapulmonary tumors and extrapulmonary metastases is still a challenge for the clinical testing. This study aims to explore the differences of PD-L1 expression in test samples obtaining from different sites of NSCLC. Read More

Oncologist 2022 Apr;27(4):299-306

Department of Oncology, Haukeland University Hospital and Department of Clinical Science, University of Bergen, Bergen, Norway.

Background: Although the gastrointestinal tract (including the pancreas, gastroenteropancreatic (GEP) is the most common site for extrapulmonary neuroendocrine carcinoma (NEC), the current treatment patterns of locoregional GEP NEC and in particular, the role of surgical resection is unclear.

Methods: Data from the National Cancer Database between 2004 and 2016 were used for this study.

Results: Of 2314 GEP NEC cases (stages I-III), 52. Read More

Mol Med Rep 2022 04 25;25(4). Epub 2022 Feb 25.

Division of Biosciences, College of Health, Medicine and Life Sciences, Brunel University London, Uxbridge UB8 3PH, UK.

In addition to the angiotensin‑converting enzyme 2 (ACE2), a number of host cell entry mediators have been identified for severe acute respiratory syndrome coronavirus‑2 (SARS‑CoV‑2), including transmembrane protease serine 4 (TMPRSS4). The authors have recently demonstrated the upregulation of TMPRSS4 in 11 different cancers, as well as its specific expression within the central nervous system using tools. The present study aimed to expand the initial observations and, using immunohistochemistry, TMPRSS4 protein expression in the gastrointestinal (GI) tract and lungs was further mapped. Read More

Case Rep Oncol 2021 Sep-Dec;14(3):1806-1813. Epub 2021 Dec 23.

Medical Oncology Department, Liverpool Hospital, Liverpool, New South Wales, Australia.

Small cell carcinoma is associated with a number of paraneoplastic syndromes. We report a case of a 42-year-old female who presented with primary laryngeal small cell carcinoma associated with concurrent paraneoplastic dermatomyositis and paraneoplastic angioedema secondary to acquired C1 esterase inhibitor deficiency. The patient required extensive treatment for her dermatomyositis including high-dose corticosteroid therapy and intravenous immunoglobulin followed by steroid-sparing disease-modifying immunosuppression. Read More

Clin Cancer Res 2022 May;28(10):1999-2019

Cancer Research UK Manchester Institute Cancer Biomarker Centre, The University of Manchester, Alderley Park, United Kingdom.

Poorly differentiated neuroendocrine carcinomas (PD-NEC) are rare cancers garnering interest as they become more commonly encountered in the clinic. This is due to improved diagnostic methods and the increasingly observed phenomenon of "NE lineage plasticity," whereby nonneuroendocrine (non-NE) epithelial cancers transition to aggressive NE phenotypes after targeted treatment. Effective treatment options for patients with PD-NEC are challenging for several reasons. Read More

J Gastrointest Oncol 2021 Dec;12(6):3148-3154

Department of Medical Oncology and Therapeutics Research, City of Hope Comprehensive Cancer Center, Duarte, CA, USA.

Extrapulmonary small cell carcinoma (EPSCC) is a rare and aggressive clinical entity that can involve a variety of anatomic locations, including the gastrointestinal tract. Involvement of the gastrointestinal tract is associated with a particularly poor prognosis with patients often presenting with widespread dissemination on initial clinical presentation or rapidly progressing to systemic disease from locoregional involvement. Primary small cell carcinoma of the anal canal is extremely rare, with limited published case reports in the literature. Read More

J Gastrointest Cancer 2022 Jan 10. Epub 2022 Jan 10.

Department of Medical Oncology, Fox Chase Cancer Center, Philadelphia, PA, USA.

Introduction: This investigation aims to assess the outcomes for second-line therapies to treat extrapulmonary neuroendocrine carcinoma (EP-NEC) after first-line platinum-based chemotherapy.

Methods: With IRB approval, we conducted a retrospective study of EP-NEC patients that progressed on first-line platinum chemotherapy from 2008 to 2018. Demographic data and treatment-related characteristics were collected and represented as descriptive statistics. Read More

Eur J Cancer 2021 12 6;159:167-173. Epub 2021 Nov 6.

Division of Hematology/Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Republic of Korea. Electronic address:

Background: Pralsetinib, an RET inhibitor, has shown a dramatic response in patients with RET fusion- or mutation-positive tumours in previous studies. As a novel target agent, however, the safety of pralsetinib remains to be determined. Herein, we present two cases of extrapulmonary tuberculosis (TB) that developed during pralsetinib therapy. Read More

Neuroendocrinology 2021 Oct 14. Epub 2021 Oct 14.

Department of Oncology, Cross Cancer Institute and the University of Alberta, Edmonton, Alberta, Canada.

Objective: This study aimed to assess the survival differences between cisplatin/etoposide versus carboplatin/etoposide chemotherapy regimens in the management of patients with extrapulmonary neuroendocrine carcinomas (NECs).

Methods: Administrative cancer care databases in the province of Alberta, Canada, were reviewed, and patients with extrapulmonary NECs (including those with small cell and large cell neuroendocrine carcinomas) who were treated with either cisplatin/etoposide or carboplatin/etoposide, 2004-2019, were reviewed. Kaplan-Meier survival estimates were used to compare the survival outcomes according to the type of platinum agent, and multivariable Cox regression analysis was used to assess the impact of the type of platinum agent on overall survival outcomes. Read More

BMJ Case Rep 2021 Oct 8;14(10). Epub 2021 Oct 8.

Department of Surgery, St Mark's Hospital Foundation, Harrow, UK.

A generally well 71-year-old man presented to his general practitioner with altered bowel habit and haematochezia. Colonoscopy revealed a malignant-appearing rectal mass, with histological features of extrapulmonary small cell carcinoma (EPSCC) of the rectum. Imaging demonstrated limited stage disease with a threatened circumferential resection margin. Read More

J Pathol Transl Med 2021 Nov 6;55(6):398-405. Epub 2021 Oct 6.

Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Background: Data on the prevalence of programmed death-ligand 1 (PD-L1) expression and tumor-infiltrating lymphocytes (TILs) in non-small cell lung cancer (NSCLC) and their clinical significance in Indian patients are limited.

Methods: Newly diagnosed NSCLC cases (adenocarcinoma or squamous cell carcinoma [SqCC] histology) were included in the present study. The TILs were evaluated based on morphology on hematoxylin and eosin-stained slides. Read More

Mod Pathol 2022 02 7;35(2):218-227. Epub 2021 Sep 7.

Key laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Pathology, Peking University Cancer Hospital & Institute, Beijing, China.

Metastatic tumors (MTs) may show different characteristics of the immune microenvironment from primary tumors (PTs) in non-small cell lung cancer (NSCLC). The heterogeneity of immune markers in metastatic NSCLC and its associated factors has not been well demonstrated. In this study, 64 surgically resected specimens of paired PTs and MTs were obtained from 28 patients with NSCLC. Read More

Prostate 2021 09 12;81(13):938-943. Epub 2021 Jul 12.

Division of Medical Oncology, Mayo Clinic, Rochester, Minnesota, USA.

Introduction: Chemotherapy in combination with immunotherapy has a proven survival benefit compared to chemotherapy alone in extensive stage small cell lung cancer (SCLC) and is the new standard of care. Since extrapulmonary small cell carcinomas (SCCs) are less common, treatment paradigms are reasonably extrapolated from SCLC regimens. We examined our institution's experience utilizing the combination of chemotherapy and immunotherapy (as used in SCLC) for SCC and neuroendocrine carcinoma of the prostate. Read More

Anticancer Drugs 2022 01;33(1):91-93

Director Medical Oncologist, Metaxa Memorial Cancer Hospital of Pireaus, Greece.

Small cell carcinomas (SCCs) are poorly differentiated neuroendocrine tumors that arise predominantly in the lung and gastrointestinal tract (GIT). Neuroendocrine carcinomas can occur in a variety of extrapulmonary sites throughout the body, including breast, larynx, GIT, prostate, urinary bladder, ovary and cervix. This is a case report of a 55-year-old Asian female patient with metastatic high-grade neuroendocrine carcinoma of unknown origin that responded notably to off-label nivolumab maintenance treatment after first-line carboplatin - etoposide chemotherapy and radiation. Read More

Am J Case Rep 2021 Jun 14;22:e932081. Epub 2021 Jun 14.

Department of Hematology-Oncology, Alabama Oncology Hematology Associates, Montgomery, AL, USA.

BACKGROUND Lurbinectedin (Lurbi) was first approved in June 2020 for metastatic small cell lung cancer (SCLC) patients with progression following platinum-based chemotherapy. Extrapulmonary small cell neuroendocrine cancers (SCNECs) are treated with regimens used for SCLCs. Tumor lysis syndrome (TLS) in solid SCLCs and SCNECs following Lurbi use has not been reported in the literature so far. Read More

Anticancer Res 2021 Jun;41(6):3023-3027

Department of Pulmonology and Critical Care, Allegheny Health Network, Pittsburgh, PA, U.S.A.

Background/aim: Small cell cancer of the esophagus (SCCE) is an extremely rare entity with an aggressive clinical course, thus early diagnosis and treatment are important for improved survival.

Case Report: A 35-year-old male presented with dysphagia, loss of appetite and weight loss. Diagnostic workup revealed an esophageal mass, which was diagnosed as primary non-Hodgkin lymphoma (NHL) on initial biopsy. Read More

Genes Dev 2021 06 20;35(11-12):870-887. Epub 2021 May 20.

Division of Human Biology, Fred Hutchinson Cancer Research Center, Seattle, Washington 98109, USA.

Small cell lung carcinoma (SCLC) is among the most lethal of all solid tumor malignancies. In an effort to identify novel therapeutic approaches for this recalcitrant cancer type, we applied genome-scale CRISPR/Cas9 inactivation screens to cell lines that we derived from a murine model of SCLC. SCLC cells were particularly sensitive to the deletion of NEDD8 and other neddylation pathway genes. Read More

J Cytol 2021 Jan-Mar;38(1):8-13. Epub 2021 Jan 8.

Department of Pathology, Dong-A University College of Medicine, Busan, Korea.

Context: Subtyping of solitary pulmonary lesion (SPL) in small amount of cytology specimen using a limited panel of immunohistochemistry (IHC) markers is very important to the correct choice of treatment. This study was performed to categorize non-small cell carcinoma-not otherwise specified (NSCC-NOS) on cytology in patients with SPL, especially with regard to the incidence of metastatic cancer.

Materials And Methods: We reviewed 91 cases, in which a precise morphology-based, lineage-specific IHC-aided subtyping was not possible, that qualified as NSCC-NOS on cytology. Read More

Int J Hyperthermia 2021 ;38(1):640-649

Department of Minimally Invasive Tumor Therapies Center, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, China.

Objectives: To explore the outcomes of CT-guided percutaneous microwave ablation (MWA) in non-small cell lung cancer (NSCLC) patients, and then develop an effective nomogram to predict the survival.

Methods: NSCLC patients treated with MWA were randomly allocated to either the training cohort or the validation cohort (3:1). The primary outcome measurement was overall survival (OS), whose predictors were identified by univariate and multivariate analyses in the training cohort. Read More

Oncology 2021 26;99(6):373-379. Epub 2021 Mar 26.

Institute of Oncology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.

Introduction: Extrapulmonary small-cell cancer (EPSCC) is a relatively rare malignancy. The management of EPSCC is usually extrapolated from small-cell lung cancer (SCLC). In spite of the morphological similarity of the 2 malignancies, there are many differences in clinical features, prognosis, and recommendations of treatment of these disorders. Read More

Cureus 2021 Jan 17;13(1):e12748. Epub 2021 Jan 17.

Hematology and Medical Oncology, Allegheny Health Network, Pittsburgh, USA.

Objectives: We conducted a retrospective analysis of patients with extrapulmonary neuroendocrine carcinomas (EPNECs) to explore the distribution and overall outcomes by different regimens and their primary sites.

Setting: We reviewed the outcomes of one of the largest data sets of patients with extrapulmonary small cell carcinomas (EPSCCs) identified at Allegheny General Hospital located in Pittsburgh, Pennsylvania, USA.

Participants: Patients diagnosed with grade 3 EPNECs were retrospectively identified. Read More

JAMA Oncol 2021 May;7(5):759-770

Department of Medical Oncology, University Medical Centre Groningen, University of Groningen, Groningen, the Netherlands.

Importance: Patients with extrapulmonary neuroendocrine carcinomas (EPNECs) receive essentially the same treatment as those with small cell lung cancer (SCLC) despite differences in origin, clinical course, and survival. This SCLC-based approach is attributable to the rarity of EPNECs, which impedes the use of randomized clinical trials. However, neuroendocrine carcinomas are becoming more common because of the increasing use of systemic cancer therapy for adenocarcinomas. Read More

Sci Transl Med 2021 01;13(578)

Developmental Therapeutics Branch, Center for Cancer Research, NCI, Bethesda, MD 20892, USA.

Because tobacco is a potent carcinogen, secondary causes of lung cancer are often diminished in perceived importance. To assess the extent of inherited susceptibility to small cell lung cancer (SCLC), the most lethal type of lung cancer, we sequenced germline exomes of 87 patients (77 SCLC and 10 extrapulmonary small cell) and considered 607 genes, discovering 42 deleterious variants in 35 cancer-predisposition genes among 43.7% of patients. Read More

Medicines (Basel) 2021 Jan 18;8(1). Epub 2021 Jan 18.

Department of Urology, SUNY Downstate Health Sciences University, 450 Clarkson Ave, P.O. Box 79, Brooklyn, NY 11203, USA.

Primary small cell carcinoma of the kidney (PSCCK) is exceedingly rare and data on disease characteristics and outcomes are sparse. This study examines a nationally-representative cancer registry to better characterize PSCCK. We queried the National Cancer Database to identify patients with histology-confirmed PSCCK from 2004 to 2015. Read More

Thorac Cancer 2021 02 14;12(3):387-390. Epub 2020 Dec 14.

Lung Cancer and Respiratory Disease Center, Yamanashi Prefectural Central Hospital, Kofu, Japan.

Extensive disease small cell lung cancer (ED-SCLC) is a systemic disease characterized by diffuse metastases and a poor prognosis. Oligometastatic cases in ED-SCLC are rare. This study reports the case of a 72-year-old Japanese male. Read More

Cancer Treat Res Commun 2020 21;25:100246. Epub 2020 Nov 21.

Juravinski Cancer Centre, 699 Concession St, Hamilton, ON, L8V 5C2, Canada; McMaster University, 1280 Main St W, Hamilton, ON, L8S 4L8, Canada.

Background: To determine if synchronous extrapulmonary malignancies in early stage lung cancer impact survival and cost of care in the current era of improved therapies and diagnostics.

Methods: Patients with stage I and II lung cancer were identified from the Ontario Cancer Registry and prognostic factors were obtained from provincial health administrative databases. Synchronous extrapulmonary malignancies were defined as those detected within 6 months from diagnosis of the lung primary. Read More

Curr Med Res Opin 2021 01 23;37(1):71-81. Epub 2020 Nov 23.

Department of Medical Oncology, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, P.R. China.

Objective: Few studies have investigated extrapulmonary small cell carcinoma (EPSCC) in a systematic way. This study is to analyze EPSCC in 11 tumor sites from different aspects in the United States (1975-2016).

Methods: In total 4397 patients diagnosed with EPSCC in 11 primary tumor locations were selected from the Surveillance, Epidemiology and End Results (SEER) database. Read More

Urol Case Rep 2020 Nov 25;33:101320. Epub 2020 Jun 25.

Division of Urologic Oncology (Fox Chase), Fox Chase Cancer Center-Temple University Health System, Philadelphia, PA, USA.

Small cell carcinoma (SCC) is an aggressive histologic subtype of neuroendocrine tumor and is most commonly of bronchogenic origin. However, it can present in an extrapulmonary fashion. Primary extrapulmonary small cell carcinoma (EPSCC) is a rare disease entity, especially within the genitourinary system and furthermore of the adrenal gland. Read More

Anticancer Res 2020 Oct;40(10):5933-5938

Division of Medical Oncology, Allegheny Health Network Cancer Institute, Pittsburgh, PA, U.S.A.

Background/aim: Primary small cell neuroendocrine carcinoma (SCNEC) of the adrenal gland is extremely rare with limited reports in the literature. There remain no definitive treatment guidelines, largely due to the rarity of the malignancy.

Case Report: We present the case of a 62-year-old Caucasian male who presented with low back pain and was found to have a large retroperitoneal mass arising from the left adrenal gland, measuring 18. Read More