Background

These syndromes are caused by hormones and proteins produced by the host tumor. It occurs in 10% of malignancies but some cancers are particularly prone to develop this syndrome.
OUTLINE

Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/ Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

SYNDROME	UNDERLYING MALIGNANCY
PARANEOPLASTIC ACRAL VASCULAR SYNDROME	Adenocarcinoma
Paraneoplastic acral vascular syndrome: epidemiologic features, clinical manifestations, and disease sequelae. Poszepczynska-Guigne E, Viguier M, Chosidow O, Orcel B, Emmerich J, Dubertret L. Department of Dermatology, Hopital Saint-Louis, 1 avenue Claude-Vellefaux, 75475 Paris Cedex 10, France.	J Am Acad Dermatol 2002 Jul;47(1):47-52 Abstract quote BACKGROUND: Acral vascular syndromes associated with malignancy have rarely been reported. OBJECTIVE: Our purpose was to assess the clinical and evolving features of paraneoplastic acral vascular syndromes. Patients and Methods: Two cases of paraneoplastic gangrene are described and analyzed together with previously reported cases identified by a MEDLINE search. RESULTS: Among the 68 patients identified, 40 had gangrene, 16 had acrocyanosis, and 12 had Raynaud's phenomenon. The male to female ratio was 0.89; median age was 59 years. Fingers were affected in 94%. Adenocarcinomas were the predominant associated malignancies (41%), and metastases were observed in 41%. The acral vascular syndromes in 48% of the patients definitively regressed after tumor treatment. Forty-four percent of the patients died within 2 years. A favorable cutaneous outcome was obtained with prostacyclin infusions in 6 patients. CONCLUSION: A neoplastic origin of acral vascular syndrome should be considered in elderly patients, especially men, in the absence of usual causative conditions.
CUSHING'S SYNDROME	Small cell carcinoma of the lung Pancreatic carcinoma Neural tumors
ERUPTIVE MELANOTIC MACULES AND PAPULES	J Cutan Pathol. 2003 Aug;30(7):463-9. Abstract quote BACKGROUND: Malignancies may be associated with paraneoplastic cutaneous manifestations, including pigmentary disorders. METHODS: The clinical findings were reviewed. Skin and tumor tissue samples were examined by routine histology, immunohistochemistry, and in one case also by electron microscopy. RESULTS: Two patients developed diffuse melanotic macules and papules associated with visceral adenocarcinoma. One patient was a 64-year-old man with advanced carcinoma of the distal esophagus. The other was a 62-year-old man with metastatic pulmonary adenocarcinoma. The detection of the primary tumor in both patients was preceded by the rapid onset of melanotic macules and papules in the anogenital region and in one patient also around both nipples. The pigmented lesions were histologically characterized by a lentiginous melanocytic proliferation of large and heavily pigmented melanocytes associated with hyperpigmentation of adjacent keratinocytes. Both patients had been misdiagnosed as having epidermotropic metastatic malignant melanoma. None of them had prior, concurrent, or subsequent cutaneous or extracutaneous invasive melanoma. Both patients died of metastatic adenocarcinoma. CONCLUSION: Eruptive melanotic macules and papules represent an under-recognized paraneoplastic syndrome. The cases illustrate a diagnostic pitfall for clinicians and pathologists unaware of this phenomenon.
	Eruptive melanotic macules and papules associated with adenocarcinoma. Busam KJ, Sachs DL, Coit DG, Halpern A, Hwu WJ. Department of Pathology, Department of Medicine, Dermatology, Department of Surgery, and Department of Medicine, Clinical Immunology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA.
HYPERCALCEMIA	Squamous cell CA of the lung Breast CA Renal CA Adult T cell leukemia/lymphoma Ovarian CA
HYPOGLYCEMIA	Fibrosarcoma Mesenchymal sarcomas Hepatocellular CA
Carcinoid syndrome	Bronchial adenoma or carcinoid Pancreatic CA Gastric CA
Polycythemia	Renal CA Cerebellar hemangioma Hepatocellular CA
Myasthenia	Brochogenic CA
CNS and PNS syndromes	Breast CA
Acanthosis nigricans	Gastric CA Lung CA Uterine CA
Dermatomyositis	Bronchogenic CA Breast CA
Hypertrophic osteoarthropathy and clubbing of the fingers	Bronchogenic CA
Venous thrombosis (Trousseau phenomenon)	Pancreatic CA Bronchogenic CA Other CA
Nonbacterial thrombotic endocarditis	Advanced CA
Anemia	Thymic neoplasms
Nephrotic syndrome	Various CA
SYNDROME OF INAPPROPRIATE ADH SECRETION (SIADH)	Small cell carcinoma of the lung Intracranial neoplasms

HISTOLOGICAL TYPES	CHARACTERIZATION
GENERAL
SKIN
Clinical and pathologic findings of paraneoplastic dermatoses. Chung VQ, Moschella SL, Zembowicz A, Liu V. Harvard Medical School, Boston, Massachusetts, USA.	J Am Acad Dermatol. 2006 May;54(5):745-62; quiz 763-6. Abstract quote   Paraneoplastic dermatoses comprise a heterogeneous group of noninherited skin conditions that manifest internal malignancy. Familiarity with paraneoplastic dermatoses is important to both clinician and pathologist alike, as recognition of such a condition offers opportunity for early diagnosis and treatment of internal malignancy; monitoring for tumor recurrence; and insight into pathophysiology which may yield possible clues to treatment. Herein are reviewed 16 of the best established paraneoplastic dermatoses that display distinctive clinical and pathologic findings. LEARNING OBJECTIVE: At the conclusion of this leaning activity, participants should be able to recognize, diagnose, and describe the clinical and pathologic findings of paraneoplastic dermatoses.

Semin Oncol 1997;24:299.
Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

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Last Updated May 9, 2006

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