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      Acantholytic squamous cell carcinoma of the lung with marked lymphogenous metastases and high titers of myeloperoxidase-antineutrophil cytoplasmic antibodies: a case report

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          Abstract

          Background

          Acantholytic squamous cell carcinoma (ASQCC), histologically characterized by intercellular bridge loosening, is recognized as a rare variant of squamous cell carcinoma (SQCC). ASQCC may demonstrate a worse prognosis than conventional SQCC. Pulmonary ASQCC is particularly rare; its biological behavior and prognostic data have not been reported.

          Case presentation

          We report the clinical and autopsy findings of a 71-year-old Japanese man with pulmonary ASQCC. Pulmonary lesions, suggestive of idiopathic interstitial pneumonia, were radiologically observed 3 and 6 years prior to the patient’s most recent hospitalization; however, the patient did not undergo further medical examinations. Upon being discovered unconscious, the patient was admitted to our hospital. Dehydration and lower limb muscle weakness were noted, as were laboratory findings of coagulation abnormalities and renal dysfunction. Computed tomography helped confirm a 21-mm peripheral nodule in the upper left lobe of the lung, with associated swollen lymph nodes in the bilateral hilar, mediastinal, and para-aortic regions. Brain and spinal lesions, suggestive of neurological disturbances, were not found. Small cell lung carcinoma was suspected, upon admission, but high serum levels of squamous cell carcinoma antigen and cytokeratin-19 fragments were present. Therefore, advanced lung cancer, possibly SQCC, was diagnosed. The patient was treated with best supportive therapy, and died one month after admission. Hypercalcemia and high serum levels of parathyroid hormone-related protein (PTHrP) and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) titers were observed. Progressive renal insufficiency was absent due to improved renal function subsequent to hydration. An autopsy helped confirm the left lung tumor as an ASQCC associated with pulmonary lymphangitic carcinomatosis and multiple metastases in the lungs and lymph nodes. Skin lesions suggesting malignant tumors were absent. The metastatic lesions consisted largely of acantholytic tumor cells, and the lungs showed usual interstitial pneumonia pattern; vasculitis was absent.

          Conclusions

          This is the first reported case of pulmonary ASQCC resulting in an aggressive clinical course, with marked lymphogenous metastases and PTHrP-associated hypercalcemia. The high serum MPO-ANCA titers were clinicopathologically insignificant, but may have been related to the pulmonary interstitial lesion. Pulmonary ASQCC represents a highly malignant subset of lung cancer.

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          Most cited references41

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          Trousseau's syndrome: multiple definitions and multiple mechanisms.

          Ajit Varki (2007)
          In 1865, Armand Trousseau noted that unexpected or migratory thrombophlebitis could be a forewarning of an occult visceral malignancy. An analysis by Sack and colleagues in 1977 extended the term Trousseau's syndrome to include chronic disseminated intravascular coagulopathy associated with microangiopathy, verrucous endocarditis, and arterial emboli in patients with cancer, often occurring with mucin-positive carcinomas. In recent times the term has been ascribed to various clinical situations, ranging all the way from these classic descriptions to any kind of coagulopathy occurring in the setting of any kind of malignancy. These multiple definitions of Trousseau's syndrome are partly the consequence of multiple pathophysiologic mechanisms that apparently contribute to the hypercoagulability associated with cancer. Even the classic syndrome probably represents a spectrum of disorders, ranging from exaggerated fluid-phased thrombosis dependent on prothrombotic agents such as tissue factor to a platelet- and endotheliumum-based selectin-dependent microangiopathy associated with mucin-producing carcinomas, along with thrombin and fibrin production. Also considered here are recent hypotheses about genetic pathways within tumor cells that might trigger these thrombotic phenomena, and the reasons why therapy with heparins of various kinds remain the preferred treatment, probably because of their salutary actions on several of the proposed pathologic mechanisms.
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            Stroke in patients with cancer: incidence and etiology.

            To assess the incidence and type of strokes in patients with cancer at Memorial Sloan-Kettering Cancer Center. Retrospective review of all ischemic strokes diagnosed by a neurologist and confirmed by neuroimaging between February 1997 and April 2001 was conducted. Age, gender, cancer diagnosis and stage, and vascular risk factors were recorded. NIH Stroke Scale and modified Rankin Scale scores were calculated retrospectively. Stroke etiology was assigned independently by two neurologists using the Trial of Org 10172 in Acute Stroke Treatment (TOAST) criteria. Ninety-six patients with a confirmed stroke were identified. The median age was 67, and 61.5% were men. The distribution of vascular risk factors was comparable with that seen in large stroke trials. Lung cancer (30%) was the most common primary tumor followed by brain and prostate cancer (9% each). Strokes were embolic in 52 (54%) and nonembolic in 44 (46%). Eleven of 12 tested patients had an elevated D-dimer level, but in only 3 patients could a definitive diagnosis of nonbacterial thrombotic endocarditis be made. The median survival was 4.5 months (95% CI 2.8 to 9.5) from the diagnosis of stroke; 25% of patients died within 30 days. Treatment had no effect on survival. Embolic strokes are the commonest cause of stroke in patients with cancer, due partially to hypercoagulability, whereas atherosclerosis accounted for only 22% of stroke in this population. Outcome was primarily determined by the underlying malignancy and the patient's neurologic condition.
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              Cerebrovascular Complications in Patients with Cancer

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                Author and article information

                Contributors
                +81-88-822-1201 , kenjiyorita@gmail.com
                stnqbwc8228@md.pikara.ne.jp
                yoko242@gmail.com
                kurochankochi@yahoo.co.jp
                majankiti@yahoo.co.jp
                ariik1130@gmail.com
                y.yoshimoto.y.1@chime.ocn.ne.jp
                nakakimi@ma.pikara.ne.jp
                s-itou@kfy.biglobe.ne.jp
                Journal
                BMC Cancer
                BMC Cancer
                BMC Cancer
                BioMed Central (London )
                1471-2407
                16 March 2018
                16 March 2018
                2018
                : 18
                : 300
                Affiliations
                [1 ]GRID grid.459719.7, Department of Diagnostic Pathology, , Japanese Red Cross Kochi Hospital, ; 2-13-51, Shinhonmachi, Kochi-city, Kochi, 780-8562 Japan
                [2 ]GRID grid.459719.7, Department of Internal Medicine, , Japanese Red Cross Kochi Hospital, ; 2-13-51, Shinhonmachi, Kochi-city, Kochi, 780-8562 Japan
                [3 ]GRID grid.459719.7, Department of Radiology, , Japanese Red Cross Kochi Hospital, ; 2-13-51, Shinhonmachi, Kochi-city, Kochi, 780-8562 Japan
                Author information
                http://orcid.org/0000-0001-6428-0222
                Article
                4218
                10.1186/s12885-018-4218-8
                5857100
                29548309
                de1bb0b9-043f-4613-b218-d80d198f84b9
                © The Author(s). 2018

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 3 July 2017
                : 13 March 2018
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2018

                Oncology & Radiotherapy
                squamous cell carcinoma,acantholytic squamous cell carcinoma,lymphogenous metastasis,small cell lung carcinoma,myeloperoxidase-antineutrophil cytoplasmic antibody,hypercalcemia,parathyroid hormone–related protein

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