We report the unique association of Masson?s pseudoangiosarcoma (endothelial papillary hyperplasia) and extramedullary  hematopoiesis. The lesion was present as a violaceous nodule on the side of the tongue of a 78-year-old man with  history of multiple myeloma and long-standing mild anemia. This association between a peculiar form of thrombus  organization and extramedullary hematopoiesis has been reported previously only once, in an infant with a cranial  hematoma, and raises interesting pathogenetic questions

Moragón Gordón, Manuel

Santonja Garriga, Carlos

Sus, Jesús de

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Repositori d'Objectes Digitals per a l'Ensenyament la Recerca i la Cultura

E556Med Oral Patol Oral Cir Bucal.  2007 Dec 1;12(8):E556-9.                                                                                                                                                                    Masson’s pseudoangiosarcoma                                                        Extramedullary hematopoiesis within endothelial papillary hyperplasia  (Masson’s pseudoangiosarcoma) of the tongueCarlos Santonja 1, Jesús de Sus 2, Manuel Moragón 3(1) MD. Department of Pathology, Fundación Jiménez Díaz. Madrid(2) MD. Department of Pathology, Hospital General de Elda. Alicante(3) MD. Department of Dermatology, Hospital Universitario de San Juan. AlicanteCorrespondence:Dr. Carlos Santonja. Servicio de Anatomía Patológica.Fundación Jiménez Díaz. Avenida de los Reyes Católicos 2. 28040 Madrid. SpainE-mail: csantonja@fjd.esReceived: 17-12-2006Accepted: 12-07-2007Santonja C, de Sus J, Moragón M. Extramedullary hematopoiesis within endothelial papillary hyperplasia  (Masson’s pseudoangiosarcoma) of the tongue. Med Oral Patol Oral Cir Bucal. 2007 Dec 1;12(8):E556-9.© Medicina Oral S. L. C.I.F. B 96689336 - ISSN 1698-6946AbStrACtWe report the unique association of Masson’s pseudoangiosarcoma (endothelial papillary hyperplasia) and extramedul-lary hematopoiesis. The lesion was present as a violaceous nodule on the side of the tongue of a 78-year-old man with history of multiple myeloma and long-standing mild anemia. This association between a peculiar form of thrombus organization and extramedullary hematopoiesis has been reported  previously only once, in an infant with a cranial hematoma, and raises interesting pathogenetic questions.Key words: Extramedullary hematopoiesis, intravascular endothelial papillary hyperplasia, tongue, thrombus organiza-tion.Indexed in: -Index Medicus / MEDLINE  /  PubMed            -EMBASE, Excerpta Medica-SCOPUS-Indice Médico Español                                                        -IBECS              Article Number: 10489719              © Medicina Oral S. L. C.I.F. B 96689336 - ISSN 1698-6946               eMail:  medicina@medicinaoral.com IntroduCtIonEndothelial papillary hyperplasia (Masson’s pseudoangio-sarcoma, vegetant intravascular hemangioendothelioma), regarded nowadays as an unusual form of  organizing thrombus (1), occurs usually as dermal or subcutaneous nodules on the head, neck, fingers and trunk. It has also been  reported in the oral cavity, with most cases involving the lower lip and the tongue (2-7). Extramedullary hema-topoiesis (i.e., the occurrence of hematopoiesis outside the bone marrow) has also been occasionally described in a variety of tumors, as an incidental finding or in the context of haematological disease (8-10).We report on  the unique association of extramedullary hematopoiesis and endothelial papillary hyperplasia  of the tongue in a patient with long-standing anemia due to multiple myeloma. CASE rEportA 78-year-old Caucasian male was seen at the Dermatology Clinic of the Hospital General de Elda (Alicante, Spain) in May of  1992 for evaluation of a violaceous nodule on the lateral tongue, which had been present for six months. In 1987 the patient had been diagnosed as having multiple myeloma stage I-A, and invasive high grade transitional cell carcinoma of the bladder, for which he had received 9 cycles of melphalan-prednisone and one cycle of onco thiotepa chemotherapy.On physical examination, a 1.5  cm rubbery violaceous nodule covered by non-ulcerated  mucosa was seen on the right lateral border of the tongue (Figure 1). A punch biopsy was performed, and two fragments of tan rubbery tissue, grossly resembling blood clot and measuring up to 0.5 cm in diameter  were obtained. The lesion   collapsed after the procedure. Microscopically, a rounded mass of fibrin (with no evidence of a vascular wall at the periphery on elastic tis-sue staining) showed an excentric irregular lumen bordered by papillary endothelial hyperplasia (Figure 2). Conspicuous foci of  extramedullary hematopoiesis were present near the lumen, consisting mostly of red cell precursors, with an admixture of myeloid cells (including rare eosinophilic E557Med Oral Patol Oral Cir Bucal.  2007 Dec 1;12(8):E556-9.                                                                                                                                                                    Masson’s pseudoangiosarcoma                                                        Fig. 1. Clinical photograph of a violaceous nodule on the right side of the tongue.Fig. 2. Microscopic low-power view of the lesion, consisting of  blood clot and loose connective tissue surrounding a  jagged-contoured lumen.Fig. 3. Coexistence of papillary endothelial hyperplasia and extramedullary hematopoiesis.E558Med Oral Patol Oral Cir Bucal.  2007 Dec 1;12(8):E556-9.                                                                                                                                                                    Masson’s pseudoangiosarcoma                                                        cells) (Figure 3). No clear-cut megacaryocytes were iden-tified. No tissue was available for further histochemical or immunohistochemical studies.Subsequent hematological evaluation  yielded the follo-wing results: Hemoglobin, 11.5 g/dL , Erythrocyte Count 3.76 x1012/L , Hematocrit 35.2% , MCV 93.7 µm3, MHC 30.7  pg/cell , MCHC 33 % Hb/cell,  Platelets 342 x109/L, Leukocytes 6.8 x 106/L (Neutrophils 0.44, Lymphocytes 0.42, Monocytes 0.08, Eosinophils 0.05, Basophils 0.05 ). No tear-drop shaped erythrocytes or erythroblasts were identified on peripheral blood films, where the percentage of plasma cells was evaluated as 3%.The patient’s clinical course was complicated by paraple-gia due to spinal  cord compression. He suffered several respiratory infections which required hospital treatment, and died at home in October of  1993,  due to bilateral bronchopneumonia.dISCuSSIonMasson’s pseudoangiosarcoma (endothelial papillary hy-perplasia), nowadays interpreted as a form of thrombus organization is usually seen as dermal or subcutaneous nodules on the head, neck, fingers and trunk. It has occa-sionally been reported in the oral cavity2 where -after the lips-  the tongue is one of the most frequent sites of oral involvement (3-7).  Although usually arising within vascular spaces (hence the designation intravascular endothelial papillary hyperplasia), some examples arising apparently in extravascular sites have been reported (11-12). In our case a vessel wall was histologically not identified, but there is indirect  evidence that the lesion lodged within a vascular space, namely the clinical site (not unusual for lingual thrombosed varices (13), the apparent collapse after biopsy, and the rounded contour of  the submitted material with an excentrically placed lumen.Exramedullary hematopoiesis (EMH) is defined as the occurrence of hematopoiesis outside the bone marrow. It is usually seen in association with haematological disorders, particularly idiopathic myelofibrosis (14) and thalassemia and other causes of  anemia. In this setting it most fre-quently involves the spleen and the liver, but many other organs   can be affected, including  the mediastinum or paraspinal region, where EMH can form tumor-like masses (15). As an isolated finding in tumors in otherwise healthy subjects, it has been reported in  an ever-growing list that includes   meningioma (16), pilomatricomas (9),  pyogenic granulomas (10),  spindle-cell lipoma (17), and  mammary fibroadenoma (18).Extramedullary hematopoiesis has been previously reported only once in association with papillary endothelial hyperpla-sia (19). In this paper, the case of a 12-day-old infant with a right middle cranial fossa hematoma was described. The resected mass consisted of thrombotic material organizing in a florid papillary pattern, in proximity to reactive fibroblasts, chronic inflammatory cells and foci of extramedullary hema-topoiesis (19). The authors hypothesized that the organizing hematoma could have been superimposed on a pre-existent vascular malformation or hemangioma.The coexistence of an organizing thrombus and EMH raises interesting pathogenetic issues. It has long been know that reorganization of  thrombi and emboli is carried out by blood mononuclear cells capable of differentiation into ma-crophages, endothelial cells and fibroblasts/myofibroblasts (20). These mononuclear cells are primarily derived from the bone marrow and include CD133/VEGFR2 endothelial progenitor cells as well as myeloid cells, and  other circula-ting progenitor cells (“side population”) (21). On the other hand, EMH has been explained by a number of theories: the splenic filtration theory (not valid for patients without a history of splenectomy), the theory that EMH is a com-pensatory phenomenon elicited by bone marrow fibrosis (which does not explain EMH in the absence of fibrosis and anemia), and the myelostimulatory theory (only applicable to organs involved in adult or fetal hematopoiesis). The most recent proposition is the “redirected differentiation theory” (22): cytokines secreted either aberrantly or in response to chronic anemia could  induce adult stem cell populations on non-hematopoietic organs  to differentiate into cells of the hematopoietic lineage.  In our case, the microenvironment of organizing thrombus seems to have harboured not only cells capable of  endo-thelial differentiation –of whichever origin- but also cells giving rise to hematopoietic elements. rEFErEnCES1. Weiss SW, Goldbum JR. Benign tumors and tumor-like lesions of blood vessels. Enzinger and Weiss’s Soft Tissue Tumors. Fourth edition. Saint Louis: Mosby; 2001. p. 877-81. 2. Heyden G, Dahl I, Angervall L. Intravascular papillary endothelial hyperplasia in the oral mucosa. Oral Surg Oral Med Oral Pathol. 1978 Jan;45(1):83-7. 3. Torne Escasany R, Umbert Millet P. Masson’s pseudoangiosarcoma of the tongue: report of two cases. J Cutan Pathol. 1985 Feb;12(1):66-71. 4. Buchner A, Merrell PW, Carpenter WM, Leider AS. Oral intravascular papillary endothelial hyperplasia. J Oral Pathol Med. 1990 Oct;19(9):419-22. 5. Tosios K, Koutlas IG, Papanicolaou SI. Intravascular papillary endo-thelial hyperplasia of the oral soft tissues: report of 18 cases and review of the literature. J Oral Maxillofac Surg. 1994 Dec;52(12):1263-8. 6. Stern Y, Braslavsky D, Shpitzer T, Feinmesser R. Papillary endothe-lial hyperplasia in the tongue: a benign lesion that may be mistaken for angiosarcoma. J Otolaryngol. 1994 Apr;23(2):81-3. 7. Ide F, Obara K, Enatsu K, Mishima K, Saito I. Rare vascular prolife-rations of the oral mucosa. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2004 Jan;97(1):75-8.  8. Green LK, Klima M, Burns TR. Extramedullary hematopoiesis occurring in a hemangioma of the skin. Arch Dermatol. 1988 Nov;124(11):1720-1. 9. Kaddu S, Beham-Schmid C, Soyer HP, Hodl S, Beham A, Kerl H. Extramedullary hematopoiesis in pilomatricomas. Am J Dermatopathol. 1995 Apr;17(2):126-30. 10. Rowlands CG, Rapson D, Morell T. Extramedullary hematopoiesis in a pyogenic granuloma. Am J Dermatopathol. 2000 Oct;22(5):434-8. 11. Chen KT. Extravascular papillary endothelial hyperplasia. J Surg Oncol. 1987 Sep;36(1):52-4. 12. Pins MR, Rosenthal DI, Springfield DS, Rosenberg AE. Florid ex-travascular papillary endothelial hyperplasia (Masson’s pseudoangiosar-coma) presenting as a soft-tissue sarcoma. Arch Pathol Lab Med. 1993 Mar;117(3):259-63. 13. Weathers DR, Fine RM. Thrombosed varix of oral cavity. Arch Der-matol. 1971 Oct;104(4):427-30. E559Med Oral Patol Oral Cir Bucal.  2007 Dec 1;12(8):E556-9.                                                                                                                                                                    Masson’s pseudoangiosarcoma                                                        14. Barosi G. Myelofibrosis with myeloid metaplasia. Hematol Oncol Clin North Am. 2003 Oct;17(5):1211-26. 15. Koch CA, Li CY, Mesa RA, Tefferi A. Nonhepatosplenic extramedu-llary hematopoiesis: associated diseases, pathology, clinical course, and treatment. Mayo Clin Proc. 2003 Oct;78(10):1223-33. 16. Gregorios JB, Bay JW, Dudley AW Jr. Extramedullary hematopoiesis in a malignant meningioma. Neurosurgery. 1983 Oct;13(4):447-51. 17. Sciot R, Bekaert J. Spindle cell lipoma with extramedullary haemato-poiesis. Histopathology. 2001 Aug;39(2):215-6. 18. Harbin LJ, Burnett S, Ghilchik M, Lee DM, Rajan P. Extramedullary haematopoiesis in a hyalinized mammary fibroadenoma. Histopathology. 2002 Nov;41(5):475-7. 19. Sickler GK, Langford LA. Intracranial tumor-forming papillary endothelial hyperplasia--a case report. Clin Neuropathol. 1990 May-Jun;9(3):125-8. 20. Leu HJ, Feigl W, Susani M, Odermatt B. Differentiation of mononuclear blood cells into macrophages, fibroblasts and endothelial cells in thrombus organization. Exp Cell Biol. 1988;56(4):201-10. 21. Urbich C, Dimmeler S. Endothelial progenitor cells: characterization and role in vascular biology. Circ Res. 2004 Aug 20;95(4):343-53. 22. Koch CA, Li CY, Mesa RA, Tefferi A. Nonhepatosplenic extramedu-llary hematopoiesis: associated diseases, pathology, clinical course, and treatment. Mayo Clin Proc. 2003 Oct;78(10):1223-33.        

Extramedullary hematopoiesis within endothelial papillary hyperplasia (Masson?s pseudoangiosarcoma) of the tongue

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Extramedullary hematopoiesis within endothelial papillary hyperplasia (Masson?s pseudoangiosarcoma) of the tongue

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