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J Clin Pathol. 1996 April; 49(4): 313–318.
PMCID: PMC500458

Aneurysmal and haemangiopericytoma-like fibrous histiocytoma.


AIM: To describe the clinicopathological features of 33 aneurysmal fibrous histiocytomas (AFH), including five cases with a haemangiopericytoma-like pattern. METHODS: Thirty three cases of AFH were studied by using routine histology and immunohistochemistry for factor XIIIa, the "cell activity marker" E9 (anti-metallothionein), NK1C3 (CD57), smooth muscle actin (SMA), factor VIII, ulex europaeus agglutinin, JC70A (CD31), and QBEND10 (CD34). The time dependent variation in histopathological features was evaluated by statistical methods (Pearson chi 2, likelihood ratio chi 2). RESULTS: Of the AFHs, 29 of 33 occurred on the extremities of adults (age range 30 to 50 years), six of which were associated with rapid growth, probably caused by trauma, and pain. Twenty one lesions were thought to be vascular and/or melanocytic lesions, including two melanomas, because of a bluish-black and/or cystic appearance. Histologically, large areas of haemorrhage, up to 50% of the tumour bulk, lacking an endothelial lining were seen in otherwise typical fibrous histiocytomas. Five cases resembled nodular stages of Kaposi's sarcoma. Variable haemosiderin deposition in histiocytes (18/33) and giant cells (11/33) was suggestive of haemosiderotic histiocytoma. A haemangiopericytoma-like pattern was seen in five otherwise indistinguishable cases. On immunohistochemistry, variable reactivity was seen for factor XIIIa (18/30), with E9 (18/30), NK1C3 (19/30), and for SMA (14/30), but labelling for vascular markers was not detected. Early lesions without iron deposition were factor XIIIa positive; late lesions with iron deposition were factor XIIIa negative. Labelling for SMA correlated with prominent sclerosis. CONCLUSION: AFHs, including a haemangiopericytoma-like variant, have a characteristic time dependent histological and immunophenotypic profile, clearly different from nodular type Kaposi's sarcoma.

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  • Santa Cruz DJ, Kyriakos M. Aneurysmal ("angiomatoid") fibrous histiocytoma of the skin. Cancer. 1981 Apr 15;47(8):2053–2061. [PubMed]
  • Zelger B, Sidoroff A, Stanzl U, Fritsch PO, Ofner D, Zelger B, Jasani B, Schmid KW. Deep penetrating dermatofibroma versus dermatofibrosarcoma protuberans. A clinicopathologic comparison. Am J Surg Pathol. 1994 Jul;18(7):677–686. [PubMed]
  • Zelger BW, Ofner D, Zelger BG. Atrophic variants of dermatofibroma and dermatofibrosarcoma protuberans. Histopathology. 1995 Jun;26(6):519–527. [PubMed]
  • Russell Jones R, Orchard G, Zelger B, Wilson Jones E. Immunostaining for CD31 and CD34 in Kaposi sarcoma. J Clin Pathol. 1995 Nov;48(11):1011–1016. [PMC free article] [PubMed]
  • Schmid KW, Ellis IO, Gee JM, Darke BM, Lees WE, Kay J, Cryer A, Stark JM, Hittmair A, Ofner D, et al. Presence and possible significance of immunocytochemically demonstrable metallothionein over-expression in primary invasive ductal carcinoma of the breast. Virchows Arch A Pathol Anat Histopathol. 1993;422(2):153–159. [PubMed]
  • Zelger B, Hittmair A, Schir M, Ofner C, Ofner D, Fritsch PO, Böcker W, Jasani B, Schmid KW. Immunohistochemically demonstrated metallothionein expression in malignant melanoma. Histopathology. 1993 Sep;23(3):257–263. [PubMed]
  • Tamada S, Ackerman AB. Dermatofibroma with monster cells. Am J Dermatopathol. 1987 Oct;9(5):380–387. [PubMed]
  • Fukamizu H, Oku T, Inoue K, Matsumoto K, Okayama H, Tagami H. Atypical ("pseudosarcomatous") cutaneous histiocytoma. J Cutan Pathol. 1983 Oct;10(5):327–333. [PubMed]
  • Leyva WH, Santa Cruz DJ. Atypical cutaneous fibrous histiocytoma. Am J Dermatopathol. 1986 Dec;8(6):467–471. [PubMed]
  • Calonje E, Mentzel T, Fletcher CD. Cellular benign fibrous histiocytoma. Clinicopathologic analysis of 74 cases of a distinctive variant of cutaneous fibrous histiocytoma with frequent recurrence. Am J Surg Pathol. 1994 Jul;18(7):668–676. [PubMed]
  • Kamino H, Jacobson M. Dermatofibroma extending into the subcutaneous tissue. Differential diagnosis from dermatofibrosarcoma protuberans. Am J Surg Pathol. 1990 Dec;14(12):1156–1164. [PubMed]
  • Schwob VS, Santa Cruz DJ. Palisading cutaneous fibrous histiocytoma. J Cutan Pathol. 1986 Dec;13(6):403–407. [PubMed]
  • Hunt SJ, Santa Cruz DJ, Miller CW. Cholesterotic fibrous histiocytoma. Its association with hyperlipoproteinemia. Arch Dermatol. 1990 Apr;126(4):506–508. [PubMed]
  • Jones RR, Spaull J, Spry C, Jones EW. Histogenesis of Kaposi's sarcoma in patients with and without acquired immune deficiency syndrome (AIDS). J Clin Pathol. 1986 Jul;39(7):742–749. [PMC free article] [PubMed]
  • Nickoloff BJ, Griffiths CE. Factor XIIIa-expressing dermal dendrocytes in AIDS-associated cutaneous Kaposi's sarcomas. Science. 1989 Mar 31;243(4899):1736–1737. [PubMed]
  • Weiss SW, Enzinger FM. Spindle cell hemangioendothelioma. A low-grade angiosarcoma resembling a cavernous hemangioma and Kaposi's sarcoma. Am J Surg Pathol. 1986 Aug;10(8):521–530. [PubMed]
  • Holden CA, Spittle MF, Jones EW. Angiosarcoma of the face and scalp, prognosis and treatment. Cancer. 1987 Mar 1;59(5):1046–1057. [PubMed]
  • Enzinger FM. Angiomatoid malignant fibrous histiocytoma: a distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm. Cancer. 1979 Dec;44(6):2147–2157. [PubMed]
  • Sanchez RL. The elusive dermatofibromas. Arch Dermatol. 1990 Apr;126(4):522–523. [PubMed]
  • Regezi JA, Nickoloff BJ, Headington JT. Oral submucosal dendrocytes: factor XIIIa+ and CD34+ dendritic cell populations in normal tissue and fibrovascular lesions. J Cutan Pathol. 1992 Oct;19(5):398–406. [PubMed]
  • Mentzel T, Calonje E, Nascimento AG, Fletcher CD. Infantile hemangiopericytoma versus infantile myofibromatosis. Study of a series suggesting a continuous spectrum of infantile myofibroblastic lesions. Am J Surg Pathol. 1994 Sep;18(9):922–930. [PubMed]
  • Zelger BW, Calonje E, Sepp N, Fink FM, Zelger BG, Schmid KW. Monophasic cellular variant of infantile myofibromatosis. An unusual histopathologic pattern in two siblings. Am J Dermatopathol. 1995 Apr;17(2):131–138. [PubMed]

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