POEMS syndrome stands for:
Epidemiology Disease Associations Pathogenesis Laboratory/Radiologic/
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Gross Appearance and Clinical Variants Histopathological Features and Variants Special Stains/
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EPIDEMIOLOGY CHARACTERIZATION SYNONYMS None INCIDENCE Rare AGE-RANGE AND MEDIAN May be in childhood SEX (MALE:FEMALE) Males favored
CHARACTERIZATION Monoclonal protein spike Level is low and usually IgG or IgA
HISTOLOGICAL TYPES CHARACTERIZATION Bone marrow Often osteosclerotic myeloma with multiple sclerotic bony lesions admixed with the plasma cells
Almost all patients have lamba light chains
SKIN Glomeruloid hemangiomas
- Solitary glomeruloid haemangioma without POEMS syndrome.
Velez D, Delgado-Jimenez Y, Fraga J.
Department of Pathology, 'La Princesa' University Hospital, Madrid, Spain.
J Cutan Pathol. 2005 Jul;32(6):449-452. Abstract quote
Background: The term 'glomeruloid haemangioma' was coined by Chan et al. for a histologically distinctive cutaneous haemangioma, which they considered a specific cutaneous marker for POEMS syndrome. Glomeruloid haemangiomas appear to be specific to POEMS syndrome, because they have not been reported in patients without this syndrome.
Methods: We report on an 86-year-old man without POEMS syndrome and with a solitary red papule on the face.
Results: A cutaneous biopsy showed histological findings consistent with a glomeruloid haemangioma. Physical examination of the skin did not show any other cutaneous lesion and laboratory and radiological studies ruled out the presence of POEMS syndrome.
Conclusions: Glomeruloid haemangiomas could exceptionally be present as solitary vascular tumours and out of the context of POEMS syndrome. To our knowledge, this is the first case reported of glomeruloid haemangioma without POEMS syndrome. Moreover, the presentation on the face is also highly unusual. Velez D, Delgado-Jimenez Y, Fraga J. Solitary glomeruloid haemangioma without POEMS syndrome.
Glomeruloid hemangioma in POEMS syndrome shows two different immunophenotypic endothelial cells
Saburo Kishimoto, Hideya Takenaka, Ryo Shibagaki, Yosuke Noda, Mari Yamamoto and Hirokazu Yasuno
Department of Dermatology, Kyoto Prefectural University of Medicine, Kyoto, Japan
J Cutan Pathol 2000;27 (2), 87-92 Abstract quote
The case of a Japanese woman with glomeruloid hemangioma, an initial marker for POEMS syndrome, is reported. Her cutaneous lesions were multiple and consisted of glomeruloid hemangiomas, cherry-type capillary hemangiomas, and a mixture of both. The specimens of glomeruloid hemangiomas were studied by paraffin section immunohistochemistry with a large panel of antibodies and electron microscopy, respectively.
The lesions, whose size ranged from minute foci to large nodules, were composed of anastomosing vascular channels resembling renal glomeruli and had irregular lumina, often featuring capillaries and sinusoid-like spaces. The vascular channels were lined by a single layer of endothelial cells, which showed two types of cells. The capillary-type endothelium possessed large vesicular nuclei with open chromatin and large amount of cytoplasm. The sinusoidal endothelium possessed small basal nuclei with dense chromatin as well as scant amount of cytoplasm. The former cells had a characteristic CD31+/CD34+/UEA I +/CD68- phenotype. Some of these cells ultrastructurally showed intracytoplasmic lumen formation. The latter cells had a characteristic CD31+/CD34-/UEA I-/CD68+ phenotype.
The present study shows that glomeruloid hemangioma has unique morphologic and immunologic features that differ from the traditional hemangiomas as well as littoral cell angioma of the spleen.
Glomeruloid hemangioma. A cutaneous marker of POEMS syndrome.
Rongioletti F, Gambini C, Lerza R.
University of Genoa, Department of Dermatology, Italy.
Am J Dermatopathol. 1994 Apr;16(2):175-8. Abstract quote
Glomeruloid hemangioma is a histologically distinctive cutaneous hemangioma that recently has been associated with POEMS syndrome.
We report a further case in which POEMS syndrome was signaled by multiple eruptive angiomas of the glomeruloid type. Histopathology showed multiple dilated vascular spaces containing a conglomerate of capillaries resulting in a structure resembling renal glomeruli. Eosinophilic globules, probably representing immunoglobulins, were found within the cytoplasm of some endothelial and stromal cells.
The finding of focal histologic features of tufted angioma (angioblastoma) in the same lesion suggests that a spectrum of hemangiomatous lesions may be seen in POEMS syndrome. Alternatively, glomeruloid hemangioma may represent a reactive endothelial proliferation rather than a neoplastic lesion.
- Glomeruloid hemangioma. A distinctive cutaneous lesion of multicentric Castleman's disease associated with POEMS syndrome.
Chan JK, Fletcher CD, Hicklin GA, Rosai J.
Department of Pathology, Yale University School of Medicine, New Haven, Connecticut 06510.
Am J Surg Pathol. 1990 Nov;14(11):1036-46. Abstract quote
A histologically distinctive cutaneous hemangioma occurring in two patients with biopsy-proven multicentric Castleman's disease associated with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) syndrome are reported. The lesions were multiple, and appeared as red to purple papules over the trunk and proximal limbs.
Microscopically, ecstatic dermal vascular spaces were seen filled with aggregates of capillaries, resulting in structures reminiscent of renal glomeruli. Interspersed between the blood-filled capillary loops were plump "stromal" cells possessing clear vacuoles and periodic acid-schiff-positive eosinophilic globules. These cells had the immunohistochemical profile of endothelial cells (positivity for factor VIII-related antigen, and negativity for leukocyte common antigen, macrophage marker, and muscle-specific actin), and probably represented immature elements that had accumulated immunoglobulins and other proteinaceous material from the circulation.
Because vascular lesions may appear before the full-blown POEMS syndrome develops, we suggest careful evaluation and follow-up of all patients presenting with glomeruloid hemangioma or cherry-type capillary hemangioma with focal glomeruloid features for potential development of this syndrome.
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. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
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Last Updated June 21, 2005
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