Variant alveolar lipoproteinosis: a syndrome with distinct clinical and pathological features.

Abstract:

:Pulmonary alveolar proteinosis (PAP) is a rare condition in which pulmonary macrophages fail to clear surfactant, resulting in the alveolar accumulation of lipoproteinaceous debris. The histopathology of PAP is typified by diffuse filling of terminal airways with eosinophilic, PAS/diastase (PAS/D)-positive acellular material. We present five patients who showed histopathological changes in the lungs consistent with mild PAP. However, these cases were notable for the abundance of degenerating alveolar macrophages, weak PAS staining of lipoproteinaceous material and paucity of lamellated bodies on ultrastructural examination. Only one patient showed the CT finding of mosaiform 'crazy-paving' and the opalescent bronchoalveolar lavage fluid characteristic of PAP. In one case, therapeutic lung lavage based on a presumptive diagnosis of PAP exacerbated respiratory distress. Three patients showed partial or near-complete resolution of disease in response to high-dose corticosteroid therapy, a treatment approach that is generally ineffective in PAP. We conclude that distinguishing 'variant alveolar lipoproteinosis' from classical PAP is clinically important. Despite the otherwise typical appearance of lipoproteinaceous alveolar material in lung biopsies, the presence of degenerating foamy macrophages and atypical histochemical, ultrastructural and radiographic features suggest a steroid-responsive form of proteinosis that is likely pathogenetically distinct and may not be amenable to whole-lung lavage.

journal_name

Pathol Int

journal_title

Pathology international

authors

Nishino M,Medoff BD,Mark EJ,Matsubara O,O'Donnell WJ,Currier PF,Kradin RL

doi

10.1111/j.1440-1827.2011.02710.x

subject

Has Abstract

pub_date

2011-09-01 00:00:00

pages

509-17

issue

9

eissn

1320-5463

issn

1440-1827

journal_volume

61

pub_type

杂志文章
  • A case of human herpesvirus-6 lymphadenitis with infectious mononucleosis-like syndrome.

    abstract::The findings of a 20 year old woman with lymphadenopathy that was probably caused by an acute human herpesvirus-6 (HHV-6) infection are reported. She clinically demonstrated various signs of acute infection such as a high fever, skin rash, liver dysfunction, leukocytosis, an elevation of the erythrocyte sedimentation ...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1995.tb03420.x

    authors: Sumiyoshi Y,Kikuchi M,Ohshima K,Takeshita M,Eizuru Y,Minamishima Y

    更新日期:1995-12-01 00:00:00

  • Experimental osteodystrophy of chronic renal failure induced by aluminum- and ferric-nitrilotriacetate in Wistar rats.

    abstract::The aluminum (AI) and iron (Fe) chelate complexes of nitrilotriacetate (NTA) cause renal insufficiency when they are administered intraperitoneally to rats. Their effects on bone metabolism were studied in 4 week old Wistar rats. Daily intraperitoneal administration of AI-NTA (3mg AI/kg for 11 weeks) induced osteomala...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1995.tb03375.x

    authors: Yukihiro S,Okada S,Takeuchi K,Inoue H

    更新日期:1995-01-01 00:00:00

  • Mucinous carcinoma of the breast: a multifaceted study with special reference to histogenesis and neuroendocrine differentiation.

    abstract::Although mucinous carcinoma (MC) of the breast is considered to originate from ductal carcinoma, it is not known whether mucinous growth begins in the intraductal carcinoma or later in the invasive carcinoma. In this study, 33 MC (16 pure without any ductal components, 10 mixed Type I with an intraductal component, se...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1046/j.1440-1827.1999.00975.x

    authors: Kato N,Endo Y,Tamura G,Katayama Y,Motoyama T

    更新日期:1999-11-01 00:00:00

  • The World Health Organization classification of malignant lymphoma: incidence and clinical prognosis in HTLV-1-endemic area of Fukuoka.

    abstract::New insights into the pathogenesis of lymphoid malignancies have been gained through novel genetic, molecular and immunological techniques. A new classification system for lymphoid malignancies, known as the new World Health Organization (WHO) classification, has been proposed recently based on these findings. The rel...

    journal_title:Pathology international

    pub_type: 杂志文章,评审

    doi:10.1046/j.1440-1827.2002.01308.x

    authors: Ohshima K,Suzumiya J,Kikuchi M

    更新日期:2002-01-01 00:00:00

  • Regular immunohistochemical localization of endothelin-1 and endothelin-B receptor in normal, hyperplastic and neoplastic human adrenocortical cells.

    abstract::The localization of endothelin (ET)-1/big ET-1, ET-3/big ET-3, ET-A and ET-B receptor was immunohistochemically examined in human adrenal glands composed of 36 normal cases, nine hyperplasia, 70 adenomas and seven carcinomas of cortical cells. In normal adrenals, ET-1/big ET-1 and ET-B receptor were regularly detected...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1997.tb03730.x

    authors: Hiraki H,Hoshi N,Hasegawa H,Tanigawa T,Emura I,Seito T,Yamaki T,Fukuda T,Watanabe K,Suzuki T

    更新日期:1997-02-01 00:00:00

  • Evaluation of multidisciplinary strategies and traditional approaches in teaching pathology in medical students.

    abstract::This study aims to evaluate the impact on the implementation of multiple strategies to improve medical student's pathology learning experience. In two consecutive years, medical students after a whole year of enrolling in pathology teaching, were invited to complete questionnaires rating and commenting on the personal...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/pin.12706

    authors: Gopalan V,Kasem K,Pillai S,Olveda D,Ariana A,Leung M,Lam AKY

    更新日期:2018-07-24 00:00:00

  • Non-neoplastic nodular lesions in the liver.

    abstract::The classical nomenclature and categorization of neoplastic and non-neoplastic nodular lesions of the liver are being revised due to the tremendous volume of information recently published on this issue. The diagnostic histopathology of non-neoplastic nodular (tumor-like) lesions of the liver that are recognizable in ...

    journal_title:Pathology international

    pub_type: 杂志文章,评审

    doi:10.1111/j.1440-1827.1995.tb03386.x

    authors: Nakanuma Y

    更新日期:1995-10-01 00:00:00

  • Histiocytoid cardiomyopathy with hypotonia in an infant.

    abstract::A 15 month old female, who had suffered from ventricular tachycardia from the prenatal period, experienced cardiac arrest at home. Once she had recovered, ventricular tachycardia occurred repeatedly. She died 7 months after admission. At autopsy, the heart showed many yellowish white nodules in the endocardium. Histol...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1995.tb03396.x

    authors: Otani M,Hoshida H,Saji T,Matsuo N,Kawamura S

    更新日期:1995-10-01 00:00:00

  • The utility of balloon enteroscopy in the diagnosis of histiocytic sarcoma of the small intestine: a case report.

    abstract::The diagnosis of the rare neoplasm histiocytic sarcoma (HS) relies on morphology and the presence of immunophenotypic features of histiocytic lineage. More than 57 cases, including 16 cases involving the gastrointestinal (GI) tract, have been described since the World Health Organization issued its classification syst...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/pin.12032

    authors: Miyazaki T,Arihiro S,Mitobe J,Aoki Y,Takikawa S,Saruta M,Matsuoka M,Koike K,Kato T,Kashiwagi H,Ikegami M,Tajiri H

    更新日期:2013-02-01 00:00:00

  • Duct-acinar-islet cell tumor of the pancreas.

    abstract::A case of a rare pancreatic tumor, duct-acinar-islet cell tumor is presented. The tumor was incidentally found in the pancreatic body on computed tomography of a 21 year old male suffering from mumps. It was well demarcated from surrounding pancreas, and spherical in shape, measured 2.5 cm in diameter. Histologic and ...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1995.tb03520.x

    authors: Okada Y,Mori H,Tsutsumi A

    更新日期:1995-09-01 00:00:00

  • Double squamous cell carcinomas, verrucous type and poorly differentiated type, of the urinary bladder unassociated with bilharzial infection.

    abstract::A case of a 66-year-old Japanese woman with two synchronous urinary bladder tumors, namely verrucous carcinoma and poorly differentiated squamous cell carcinoma, is described. Both tumors were accompanied by widespread squamous metaplasia in the background and unassociated with bilharzial infection. The poorly differe...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1997.tb04558.x

    authors: Oida Y,Yasuda M,Kajiwara H,Onda H,Kawamura N,Osamura RY

    更新日期:1997-09-01 00:00:00

  • Environmental and genetic risk factors in early human atherogenesis: lessons from the PDAY study. Pathobiological Determinants of Atherosclerosis in Youth.

    abstract::A multi-institutional study 'Pathobiological Determinants of Atherosclerosis in Youth' (PDAY) was initiated to document the natural history of atherosclerosis, its relationship to risk factors, and pathobiology of lesion development in young subjects. Pathology laboratories in nine centers collected arteries and tissu...

    journal_title:Pathology international

    pub_type: 杂志文章,多中心研究,评审

    doi:10.1111/j.1440-1827.1995.tb03476.x

    authors: Strong JP,Malcom GT,Oalmann MC

    更新日期:1995-06-01 00:00:00

  • An immunohistochemical and clinicopathological study of gastrointestinal stromal tumors.

    abstract::Immunohistochemical and clinicopathological features of 58 gastrointestinal stromal tumors (GIST) were studied. One occurred in the esophagus, 41 in the stomach, nine in the small intestine, and seven in the large intestine. By using indirect immunoperoxidase staining for Cajal cell markers (c-kit protein and CD34), s...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1046/j.1440-1827.1999.00947.x

    authors: Tazawa K,Tsukada K,Makuuchi H,Tsutsumi Y

    更新日期:1999-09-01 00:00:00

  • No evidence of a correlation between BCL10 expression and API2-MALT1 gene rearrangement in ocular adnexal MALT lymphoma.

    abstract::In the present study, 62 cases of ocular adnexal lymphoproliferative disorders were reviewed clinicopathologically. Of them, 51 were extranodal marginal zone B-cell lymphoma (MALT lymphoma), five were diffuse large B-cell lymphoma (DLBCL), one was peripheral T-cell lymphoma, one was NK/T cell lymphoma, nasal type, and...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.2004.01580.x

    authors: Adachi A,Tamaru J,Kaneko K,Kuroda H,Miura I,Kojima T,Hoshino S,Ichinohasama R,Itoyama S

    更新日期:2004-01-01 00:00:00

  • Immunohistochemical analysis of nestin and c-kit and their significance in pancreatic tumors.

    abstract::The purpose of the present study was to clarify the difference of expression of two stem cell markers, nestin and c-kit, among various pancreatic epithelial tumors and evaluate their utility. Immunohistochemistry was done for 99 surgically resected pancreatic tumor specimens, including 20 ductal adenocarcinoma (DAC), ...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.2007.02143.x

    authors: Ohike N,Sato M,Hisayuki T,Imataka H,Sato S,Wada Y,Saito K,Takahashi M,Tajiri T,Kunimura T,Morohoshi T

    更新日期:2007-09-01 00:00:00

  • Immunohistochemical study of necrotizing lymphadenitis: a possible mechanism for apoptosis involving perforin and granzyme-producing cytotoxic T cells.

    abstract::Twelve lymph node specimens with necrotizing lymphadenitis and which had florid necrotic lesions were studied immunohistochemically. The majority of viable lymphoid cells in the necrotic foci were CD8+ lymphocytes and KP1+ or PGM1+ phagocytizing macrophages. The CD8+ T cells were Leu1+, Leu2+, Leu3-, Leu4+, Leu5b+, Le...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1997.tb04432.x

    authors: Mori N,Yatabe Y,Asai J,Nagasawa T

    更新日期:1997-01-01 00:00:00

  • Endometrial low-grade stromal sarcoma with ovarian sex cord-like differentiation: report of two cases with an immunohistochemical and flow cytometric study.

    abstract::Two cases of endometrial low-grade stromal sarcoma with ovarian sex cord-like differentiation occurring in a 39-year-old woman and a 42-year-old woman are presented. Both tumors, which were intramyometrial and measured 7.5 cm and 7.0 cm in greatest diameter, respectively, showed a multinodular, ill-demarcated, and yel...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1997.tb04516.x

    authors: Fukunaga M,Miyazawa Y,Ushigome S

    更新日期:1997-06-01 00:00:00

  • Ileal carcinoid tumor complicating carcinoid heart disease and secondary retroperitoneal fibrosis.

    abstract::A 70-year-old man with metastatic liver tumors showed carcinoid syndrome with clinical symptoms of facial flushing, palpitation, dyspnea, and an itching sensation. Regurgitation of the tricuspid and aortic valves was observed by echocardiography. An elevated serum level of serotonin and a high urine excretion of serot...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1046/j.1440-1827.2000.01053.x

    authors: Sakai D,Murakami M,Kawazoe K,Tsutsumi Y

    更新日期:2000-05-01 00:00:00

  • The prognostic significance of N-myc downregulated gene 1 in lung adenocarcinoma.

    abstract::It has been reported that N-myc downstream regulated gene 1 (NDRG1) is related to the prognosis of non-small cell lung cancer (NSCLC), and associated with c-Myc degradation in NSCLC cell lines. However, the relationship of NDRG1 to prognosis or c-Myc expression in lung adenocarcinoma has not been well clarified. The p...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/pin.12644

    authors: Dai T,Dai Y,Murata Y,Husni RE,Nakano N,Sakashita S,Noguchi M

    更新日期:2018-04-01 00:00:00

  • Coexpression of hepatocyte growth factor and c-Met proto-oncogene product in synovial sarcoma.

    abstract::Hepatocyte growth factor (HGF) is a heterodimeric polypeptide growth factor that has pleiotropic roles, including those of mitogen, motogen and morphogen. The HGF receptor is characterized as a c-Met proto-oncogene product (c-Met), which is a heterodimeric tyrosine kinase receptor. Hepatocyte growth factor acts as a m...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1998.tb03836.x

    authors: Motoi T,Ishida T,Kuroda M,Horiuchi H,Oka T,Matsumoto K,Nakamura T,Machinami R

    更新日期:1998-10-01 00:00:00

  • Adenocarcinoma of small intestinal type in retroperitoneal mature teratoma.

    abstract::We report a case of small intestinal type adenocarcinoma arising in retroperitoneal mature cystic teratoma in a young male. The patient initially experienced intermittent abdominal pain, and radiographic workup revealed an encapsulated retroperitoneal mass. A laparotomy was performed and the resected specimen represen...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.2010.02582.x

    authors: Terado Y,Kurata A,Ishida T,Imamura T,Sakamoto A

    更新日期:2010-10-01 00:00:00

  • Case of mesenchymal tumor with the PPP6R3-USP6 fusion, possible nodular fasciitis with malignant transformation.

    abstract::Nodular fasciitis (NF) is a self-limiting benign disease that is characterized by rapid proliferation of fibroblastic and myofibroblastic cells. The characteristic gene fusion containing the USP6 gene is a genetic hallmark of NF and MYH9-USP6 is the most frequent fusion, suggesting that NF is not a reactive condition ...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/pin.12851

    authors: Teramura Y,Yamazaki Y,Tanaka M,Sugiura Y,Takazawa Y,Takeuchi K,Nakayama T,Kaneko T,Musha Y,Funauchi Y,Ae K,Matsumoto S,Nakamura T

    更新日期:2019-12-01 00:00:00

  • Ceroid pigment deposition in circulating blood monocytes and T lymphocytes in Hermansky-Pudlak syndrome: an ultrastructural study.

    abstract::An electron microscopic study of peripheral leukocytes obtained from a 39 year old woman with Hermansky-Pudlak syndrome was performed. Ceroid pigment granules were found within the lysosomes in 3.5% of monocytes and 5.4% of lymphocytes. Infrequently, pigment granules were also found in the parallel tubular arrays of l...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1995.tb03407.x

    authors: Sakuma T,Monma N,Satodate R,Satoh T,Takeda R,Kuriya S

    更新日期:1995-11-01 00:00:00

  • Mucosa-associated lymphoid tissue lymphoma coexisting with epithelioid granulomas in the stomach of a patient with systemic sarcoidosis.

    abstract::Malignant lymphoma arising in the stomach of a 23-year-old Japanese man with systemic sarcoidosis is presented. The patient was followed because of systemic sarcoidosis involving the lungs, eyes, and lymph nodes. Biopsy specimens from the stomach were repeated because of recurrent epigastralgia and multiple ulceration...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1997.tb03720.x

    authors: Fukuda T,Sato K,Tachikawa S,Ohnuki K,Ohtani H,Suzuki T

    更新日期:1997-12-01 00:00:00

  • Mixed pleomorphic lobular and apocrine carcinoma of the breast: A case report suggesting pathogenesis.

    abstract::Pleomorphic lobular carcinoma (PLC) of the breast is a variant of lobular carcinoma, characterized by loss of E-cadherin expression and high-grade morphologies. Whether the pathogenesis of PLC is in the ductal or the lobular lineage has been discussed. In this report, a case of PLC combined with apocrine carcinoma is ...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/pin.12781

    authors: Ishii A,Oishi T,Kakuda Y,Yasui H,Kawata T,Muramatsu K,Takahashi K,Sugino T

    更新日期:2019-05-01 00:00:00

  • Pericardial malignant mesothelioma: case report and discussion of immunohistochemical and histochemical findings.

    abstract::An extremely rare case of malignant mesothelioma of the pericardium is reported. The tumor appeared to be the biphasic type of diffuse malignant mesothelioma which present many small to fine lipid droplets in the cytoplasm, particularly that of the epithelial cell, and a positive immunohistochemical reaction with anti...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1995.tb03513.x

    authors: Ohmori T,Arita N,Okada K,Kondo M,Tabei R

    更新日期:1995-08-01 00:00:00

  • Pituicytoma with unusual histological features.

    abstract::Pituicytoma is a rare low-grade glial neoplasm that originates in the neurohypophysis or infundibulum. Because of its rare occurrence, the morphology and differentiation of pituicytoma have not been fully clarified. Here, we report a case of pituicytoma with unusual histological features mimicking ependymoma, but exhi...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.2011.02708.x

    authors: Kwon MJ,Suh YL

    更新日期:2011-10-01 00:00:00

  • Biphasic intra-abdominal desmoplastic small cell tumor in a patient with proximal spinal muscular atrophy.

    abstract::A case is reported of intra-abdominal desmoplastic small cell tumor (IDSCT) with biphasic histologic features in a patient with proximal spinal muscular atrophy. The tumor was composed of small epithelial cell nests with spindle cell sarcomatous areas. Both areas were surrounded by a desmoplastic stroma. Immunohistoch...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1996.tb03533.x

    authors: Motoyama T,Maejima T,Aizawa K,Fukuda T,Watanabe H

    更新日期:1996-01-01 00:00:00

  • Histological features of palatine tonsils in pustulosis palmaris et plantaris: a morphometric study.

    abstract::The morphological expression of the immune response of palatine tonsils obtained from patients with pustulosis palmaris et plantaris (PPP) was investigated by morphometry in conjunction with immunohistochemistry. First, to differentiate the histological features of tonsils between PPP and habitual tonsillitis, each ex...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.1994.tb02591.x

    authors: Sakai T,Kawaguchi M,Ishizawa S,Kaji T,Kitagawa K,Koizumi F

    更新日期:1994-03-01 00:00:00

  • Primary pulmonary choriocarcinoma combined with adenocarcinoma.

    abstract::An extremely rare autopsy case of primary pulmonary choriocarcinoma combined with adenocarcinomatous components in a 77-year-old Japanese man is described. The patient died of rapidly progressive respiratory dysfunction without ante-mortem diagnosis. Autopsy revealed necro-hemorrhagic areas of the primary lung tumor w...

    journal_title:Pathology international

    pub_type: 杂志文章

    doi:10.1111/j.1440-1827.2006.01977.x

    authors: Yamamoto S,Tanaka H,Takeo H,Yasuda K,Mastukuma S

    更新日期:2006-07-01 00:00:00