Pulmonary Tumor Thrombotic Microangiopathy Secondary to Extramammary Paget’s Disease: An Autopsy Case Report and Literature Review

Abstract Pulmonary tumor thrombotic microangiopathy is a fatal disease secondary to some malignant tumors, such as gastric cancer, esophageal cancer, and pancreatic cancer. A 61-year-old man presented to our clinic with a red plaque with erosion on his scrotum and was diagnosed with extramammary Pag...

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Published inCase Reports in Oncology Vol. 14; no. 3; pp. 1328 - 1332
Main Authors Kato, Akihiro, Kato, Hiroshi, Komori, Satoshi, Nakano, Satsuki, Murase, Takayuki, Nakamura, Motoki, Morita, Akimichi
Format Journal Article
LanguageEnglish
Published Basel, Switzerland S. Karger AG 16.09.2021
Karger Publishers
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ISSN1662-6575
1662-6575
DOI10.1159/000515262

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Abstract Abstract Pulmonary tumor thrombotic microangiopathy is a fatal disease secondary to some malignant tumors, such as gastric cancer, esophageal cancer, and pancreatic cancer. A 61-year-old man presented to our clinic with a red plaque with erosion on his scrotum and was diagnosed with extramammary Paget’s disease. Seven years after the initial diagnosis, multiple bone metastases were discovered, and he was started on oral administration of tegafur/gimeracil/oteracil. Two years after beginning the oral drug administration, the patient complained of dyspnea. A chest CT scan showed ground-glass opacity. Pulmonary artery blood cytology revealed carcinoma cells. He was diagnosed with pulmonary tumor thrombotic microangiopathy. At 9 days after the diagnosis, he died of circulatory and respiratory failure. The autopsy revealed microscopic metastatic tumor emboli in multiple pulmonary vessels with fibrin thrombus, which confirmed the earlier cytologic diagnosis. Observations in our case were consistent with the typical pattern of this pathology in the lung with multiple metastases. Pulmonary tumor thrombotic microangiopathy with a primary disease of cutaneous malignancy is extremely rare. Here, we report a rare case of pulmonary tumor thrombotic microangiopathy secondary to extramammary Paget’s disease with a literature review.
AbstractList Pulmonary tumor thrombotic microangiopathy is a fatal disease secondary to some malignant tumors, such as gastric cancer, esophageal cancer, and pancreatic cancer. A 61-year-old man presented to our clinic with a red plaque with erosion on his scrotum and was diagnosed with extramammary Paget's disease. Seven years after the initial diagnosis, multiple bone metastases were discovered, and he was started on oral administration of tegafur/gimeracil/oteracil. Two years after beginning the oral drug administration, the patient complained of dyspnea. A chest CT scan showed ground-glass opacity. Pulmonary artery blood cytology revealed carcinoma cells. He was diagnosed with pulmonary tumor thrombotic microangiopathy. At 9 days after the diagnosis, he died of circulatory and respiratory failure. The autopsy revealed microscopic metastatic tumor emboli in multiple pulmonary vessels with fibrin thrombus, which confirmed the earlier cytologic diagnosis. Observations in our case were consistent with the typical pattern of this pathology in the lung with multiple metastases. Pulmonary tumor thrombotic microangiopathy with a primary disease of cutaneous malignancy is extremely rare. Here, we report a rare case of pulmonary tumor thrombotic microangiopathy secondary to extramammary Paget's disease with a literature review.
Pulmonary tumor thrombotic microangiopathy is a fatal disease secondary to some malignant tumors, such as gastric cancer, esophageal cancer, and pancreatic cancer. A 61-year-old man presented to our clinic with a red plaque with erosion on his scrotum and was diagnosed with extramammary Paget's disease. Seven years after the initial diagnosis, multiple bone metastases were discovered, and he was started on oral administration of tegafur/gimeracil/oteracil. Two years after beginning the oral drug administration, the patient complained of dyspnea. A chest CT scan showed ground-glass opacity. Pulmonary artery blood cytology revealed carcinoma cells. He was diagnosed with pulmonary tumor thrombotic microangiopathy. At 9 days after the diagnosis, he died of circulatory and respiratory failure. The autopsy revealed microscopic metastatic tumor emboli in multiple pulmonary vessels with fibrin thrombus, which confirmed the earlier cytologic diagnosis. Observations in our case were consistent with the typical pattern of this pathology in the lung with multiple metastases. Pulmonary tumor thrombotic microangiopathy with a primary disease of cutaneous malignancy is extremely rare. Here, we report a rare case of pulmonary tumor thrombotic microangiopathy secondary to extramammary Paget's disease with a literature review.Pulmonary tumor thrombotic microangiopathy is a fatal disease secondary to some malignant tumors, such as gastric cancer, esophageal cancer, and pancreatic cancer. A 61-year-old man presented to our clinic with a red plaque with erosion on his scrotum and was diagnosed with extramammary Paget's disease. Seven years after the initial diagnosis, multiple bone metastases were discovered, and he was started on oral administration of tegafur/gimeracil/oteracil. Two years after beginning the oral drug administration, the patient complained of dyspnea. A chest CT scan showed ground-glass opacity. Pulmonary artery blood cytology revealed carcinoma cells. He was diagnosed with pulmonary tumor thrombotic microangiopathy. At 9 days after the diagnosis, he died of circulatory and respiratory failure. The autopsy revealed microscopic metastatic tumor emboli in multiple pulmonary vessels with fibrin thrombus, which confirmed the earlier cytologic diagnosis. Observations in our case were consistent with the typical pattern of this pathology in the lung with multiple metastases. Pulmonary tumor thrombotic microangiopathy with a primary disease of cutaneous malignancy is extremely rare. Here, we report a rare case of pulmonary tumor thrombotic microangiopathy secondary to extramammary Paget's disease with a literature review.
Abstract Pulmonary tumor thrombotic microangiopathy is a fatal disease secondary to some malignant tumors, such as gastric cancer, esophageal cancer, and pancreatic cancer. A 61-year-old man presented to our clinic with a red plaque with erosion on his scrotum and was diagnosed with extramammary Paget’s disease. Seven years after the initial diagnosis, multiple bone metastases were discovered, and he was started on oral administration of tegafur/gimeracil/oteracil. Two years after beginning the oral drug administration, the patient complained of dyspnea. A chest CT scan showed ground-glass opacity. Pulmonary artery blood cytology revealed carcinoma cells. He was diagnosed with pulmonary tumor thrombotic microangiopathy. At 9 days after the diagnosis, he died of circulatory and respiratory failure. The autopsy revealed microscopic metastatic tumor emboli in multiple pulmonary vessels with fibrin thrombus, which confirmed the earlier cytologic diagnosis. Observations in our case were consistent with the typical pattern of this pathology in the lung with multiple metastases. Pulmonary tumor thrombotic microangiopathy with a primary disease of cutaneous malignancy is extremely rare. Here, we report a rare case of pulmonary tumor thrombotic microangiopathy secondary to extramammary Paget’s disease with a literature review.
Author Kato, Akihiro
Murase, Takayuki
Nakamura, Motoki
Nakano, Satsuki
Kato, Hiroshi
Komori, Satoshi
Morita, Akimichi
AuthorAffiliation b Department of Dermatology, Kyoto Prefectural University of Medicine Graduate School of Medical Science, Kyoto, Japan
c Department of Pathology and Molecular Diagnostics, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
a Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
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Issue 3
Keywords Pulmonary tumor thrombotic microangiopathy
Cutaneous malignancy
Extramammary Paget’s disease
Autopsy
Skin cancer
Language English
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References Banno A, Chiba K, Kasai H, Nagami K. Ante-mortem diagnosis of pulmonary tumor thrombotic microangiopathy in a patient with unrecognized extramammary paget’s disease. BMJ Case Rep. 2016 Aug;bcr2016216666.
Yamauchi Y, Nakano K, Miyagawa I, Inaba Y, Nawata A, Sato N, . An autopsy case of a patient with systemic sclerosis who developed marked pulmonary hypertension because of pulmonary tumor thrombotic microangiopathy caused by gastric carcinoma. Mod Rheumatol Case Rep. 2020 Jan;4(1):56–62.
Uruga H, Fujii T, Kurosaki A, Hanada S, Takaya H, Miyamoto A, . Pulmonary tumor thrombotic microangiopathy: a clinical analysis of 30 autopsy cases. Intern Med. 2013;52(12):1317–23.
Tanaka M, Takahashi K, Kurihara Y, Yamamoto-Rikitake M, Ogusu S, Hirakawa H, . Signet ring cell carcinoma of unknown primary complicated with pulmonary tumor thrombotic microangiopathy and Krukenberg tumor. Case Rep Oncol. 2018 Jun;11(2):412–7.
Kato H, Watanabe S, Kariya K, Nakamura M, Morita A. Efficacy of low-dose 5-fluorouracil/cisplatin therapy for invasive extramammary Paget’s disease. J Dermatol. 2018 May;45(5):560–3.
Wakabayashi Y, Iwaya M, Akita M, Takeuchi W, Yamazaki K, Iijima A. Pulmonary tumor thrombotic microangiopathy caused by urothelial carcinoma expressing vascular endothelial growth factor, platelet-derived growth factor, and osteopontin. Intern Med. 2016;55(6):651–6.
Kato H, Nakamura M, Watanabe S, Oda T, Morita A. Combined serum carcinoembryonic antigen and cytokeratin 19 fragment levels provide a sensitive biomarker for lymph node metastasis in extramammary Paget’s disease. J Dermatol. 2020 Jul;47(7):763–9.
Higashi A, Dohi Y, Uraoka N, Sentani K, Uga S, Kinoshita H, . The potential role of inflammation associated with interaction between osteopontin and CD44 in a case of pulmonary tumor thrombotic microangiopathy caused by breast cancer. Intern Med. 2015;54(22):2877–80.
Oyama Y, Nishida H, Kondo Y, kusaba T, Kadowaki H, Harada T, . Pulmonary tumor thrombotic microangiopathy associated with extramammary paget’s disease: an autopsy case report. Pathol Int. 2020 Sep;70(9):680–5.
Ohara K, Fujisawa Y, Yoshino K, Kiyohara Y, Kadono T, Murata Y, . A proposal for a TNM staging system for extramammary paget’s disease: retrospective analysis of 301 patients with invasive primary tumors. J Dermatol Sci. 2016 Sep;83(3):234–9.
von Herbay A, Illes A, Waldherr R, Otto HF. Pulmonary tumor thrombotic microangiopathy with pulmonary hypertension. Cancer. 1990 Aug;66(3):587–92.
Imakura T, Tezuka T, Inayama M, Miyamoto R, Abe A, Otsuka K, . A long-term survival case of pulmonary tumor thrombotic microangiopathy due to gastric cancer confirmed by the early diagnosis based on a transbronchial lung biopsy. Intern Med. 2020;59(13):1621–7.
References_xml – reference: Kato H, Watanabe S, Kariya K, Nakamura M, Morita A. Efficacy of low-dose 5-fluorouracil/cisplatin therapy for invasive extramammary Paget’s disease. J Dermatol. 2018 May;45(5):560–3.
– reference: Kato H, Nakamura M, Watanabe S, Oda T, Morita A. Combined serum carcinoembryonic antigen and cytokeratin 19 fragment levels provide a sensitive biomarker for lymph node metastasis in extramammary Paget’s disease. J Dermatol. 2020 Jul;47(7):763–9.
– reference: Yamauchi Y, Nakano K, Miyagawa I, Inaba Y, Nawata A, Sato N, . An autopsy case of a patient with systemic sclerosis who developed marked pulmonary hypertension because of pulmonary tumor thrombotic microangiopathy caused by gastric carcinoma. Mod Rheumatol Case Rep. 2020 Jan;4(1):56–62.
– reference: Banno A, Chiba K, Kasai H, Nagami K. Ante-mortem diagnosis of pulmonary tumor thrombotic microangiopathy in a patient with unrecognized extramammary paget’s disease. BMJ Case Rep. 2016 Aug;bcr2016216666.
– reference: von Herbay A, Illes A, Waldherr R, Otto HF. Pulmonary tumor thrombotic microangiopathy with pulmonary hypertension. Cancer. 1990 Aug;66(3):587–92.
– reference: Tanaka M, Takahashi K, Kurihara Y, Yamamoto-Rikitake M, Ogusu S, Hirakawa H, . Signet ring cell carcinoma of unknown primary complicated with pulmonary tumor thrombotic microangiopathy and Krukenberg tumor. Case Rep Oncol. 2018 Jun;11(2):412–7.
– reference: Wakabayashi Y, Iwaya M, Akita M, Takeuchi W, Yamazaki K, Iijima A. Pulmonary tumor thrombotic microangiopathy caused by urothelial carcinoma expressing vascular endothelial growth factor, platelet-derived growth factor, and osteopontin. Intern Med. 2016;55(6):651–6.
– reference: Imakura T, Tezuka T, Inayama M, Miyamoto R, Abe A, Otsuka K, . A long-term survival case of pulmonary tumor thrombotic microangiopathy due to gastric cancer confirmed by the early diagnosis based on a transbronchial lung biopsy. Intern Med. 2020;59(13):1621–7.
– reference: Oyama Y, Nishida H, Kondo Y, kusaba T, Kadowaki H, Harada T, . Pulmonary tumor thrombotic microangiopathy associated with extramammary paget’s disease: an autopsy case report. Pathol Int. 2020 Sep;70(9):680–5.
– reference: Ohara K, Fujisawa Y, Yoshino K, Kiyohara Y, Kadono T, Murata Y, . A proposal for a TNM staging system for extramammary paget’s disease: retrospective analysis of 301 patients with invasive primary tumors. J Dermatol Sci. 2016 Sep;83(3):234–9.
– reference: Uruga H, Fujii T, Kurosaki A, Hanada S, Takaya H, Miyamoto A, . Pulmonary tumor thrombotic microangiopathy: a clinical analysis of 30 autopsy cases. Intern Med. 2013;52(12):1317–23.
– reference: Higashi A, Dohi Y, Uraoka N, Sentani K, Uga S, Kinoshita H, . The potential role of inflammation associated with interaction between osteopontin and CD44 in a case of pulmonary tumor thrombotic microangiopathy caused by breast cancer. Intern Med. 2015;54(22):2877–80.
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Snippet Abstract Pulmonary tumor thrombotic microangiopathy is a fatal disease secondary to some malignant tumors, such as gastric cancer, esophageal cancer, and...
Pulmonary tumor thrombotic microangiopathy is a fatal disease secondary to some malignant tumors, such as gastric cancer, esophageal cancer, and pancreatic...
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SubjectTerms Antigens
Autopsies
autopsy
Blood clots
Blood vessels
Breast cancer
Case Report
Case reports
Cellular biology
Chemotherapy
cutaneous malignancy
Cytokeratin
Dyspnea
Embolisms
Esophageal cancer
extramammary paget’s disease
Gastric cancer
Growth factors
Literature reviews
Lung cancer
Lymphatic system
Medical prognosis
Metastasis
Neoplasms. Tumors. Oncology. Including cancer and carcinogens
Pancreatic cancer
Patients
Pulmonary arteries
Pulmonary hypertension
pulmonary tumor thrombotic microangiopathy
RC254-282
Respiratory failure
Scintigraphy
Signal transduction
Skin cancer
Tumors
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Title Pulmonary Tumor Thrombotic Microangiopathy Secondary to Extramammary Paget’s Disease: An Autopsy Case Report and Literature Review
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