[ Main Page | Editorial Board | About | Instructions ]
[ Table of Contents | Archive | Archive Search | Online Submission | Sponsor | E-mail ]

Turkish Journal of Cancer
2008, Volume 38, Number 1, Page(s) 030-033
[ Summary ] [ PDF ] [ Similar Articles ] [ Mail to Editor ]
Multicentric spindle cell hemangioendothelioma of spleen: Case report
SELÇUK MEVLUT HAZİNEDAROĞLU1, HÜSEYİN AYHAN KAYAOĞLU2, FERDA DÜLGER1, EBRU SERİNSÖZ3, IŞINSU KUZU3, NEZİH ERVERDİ1
1Ankara University Faculty of Medicine, Department of General Surgery, Ankara
2Gaziosmanpaşa University Faculty of Medicine, Department of General Surgery, Tokat
3Ankara University Faculty of Medicine, Department of Pathology, Ankara-Turkey
Keywords: Hemangioendothelioma, spleen, cholecystolithiasis, hyperbilirubinemia, anemia, splenectomy, cholecystectomy
Summary
Malignant mesenchymal tumors of the spleen are rare. The vast majority of primary sarcomas of the spleen are of vascular origin. Hemangioendothelioma, a vascular tumor, is characterized by mild cellular atypia, low mitotic rate and intermediate appearance between hemangioma and conventional angiosarcoma histopathologically. We report herein a case of multicentric splenic hemangioendothelioma presenting together with symptomatic cholecystolithiasis, mild anemia and indirect hyperbilirubinemia, which were recovered after cholecystectomy and splenectomy. [Turk J Cancer 2008;38(1):30-33]
  • Top
  • Summary
  • Introduction
  • Case Presentation
  • Disscussion
  • References
  • Introduction
    Primary malignant mesenchymal tumors of the spleen are rare and highly aggressive neoplasms with a survival rate of less than one year[1]. Although hemangioendothelioma is a vascular tumor, it is characterized by mild cellular atypia and low mitotic rate; an intermediate appearance between hemangioma and conventional angiosarcoma histopathologically[2].

    According to our knowledge, this is the second case of multicentric splenic hemangioendothelioma and is the first case presenting together with cholecystolithiasis, anemia and mild indirect hyperbilirubinemia[3].

  • Top
  • Introduction
  • Case Presentation
  • Disscussion
  • References
  • Case Presentation
    A 24 year-old woman presented with right upper quadrant pain and fatigue. Physical examination was normal except mild tenderness in right upper quadrant. Laboratory investigation revealed normocytic normochromic anemia (hemoglobin, 11.4 g/dl; normal, 12-16 g/dl) and indirect hyperbilirubinemia (0.9 mg/dl; normal, 0.0-0.7 mg/dl). Abdominal ultrasound revealed multiple milimetric stones in the gallbladder and a suspicious mass in spleen. Magnetic resonance imaging (MRI) of upper abdomen highlighted this mass as a 4 cm tumor in the upper pole of spleen. There was no evidence of metastasis. The patient underwent cholecystectomy and splenectomy through a midline incision and the operation was straightforward. There was no postoperative complication and her hemoglobin and indirect bilirubin values were within the normal limits during the follow-up in the first, sixth and twelfth months after the surgery. There was no evidence of recurrence in the control abdominal computerized tomography after one year.

    Histopathological findings
    On macroscopic examination, the spleen was measured to be 15x9x4.5 cm in diameter with a normal configuration. Two distinct non-encapsulated nodules 2 cm away from each other with the diameters of 20x20x10 mm and 9x7x4 mm were detected beneath the capsule on the examination of splenic cross sections. The rest of the spleen was normal. Microscopically, the tumor was composed of vascular channels lined by plump or spindle endothelial cells and solid tumor cells in between these channels simulating hemangiopericytoma (Figure 1A, B). On high power examination, some cells of the solid areas showed cytoplasmic vacuolization or spaces partially containing erythrocytes. There were few atypical cells both among the space lining cells and in the solid areas (Figure 2A, B). However, despite this atypical appearance of the cells, the number of mitotic figures was low. Thin reticulin fibers surrounding individual and group of cells examined on reticulin staining. The tumors revealed an immunohistochemical profile for factor VIII-RA (Figure 3) and CD34 consistent with the immunophenotype of endothelial origin (Figure 4).

    Fig 1: (A,B). (A): Tumor composed of vascular channels lined by plump or spindle endothelial cells and solid tumor cells in between these channels simulating hemangiopericytoma (H&E, x40), (B): On high power examination, some cells of the solid areas showed cytoplasmic vacuolization or cytoplasmic spaces some containing erythrocytes (H&E, x100)

    Fig 2: (A,B). There were few atypical cells both among the space lining cells and in the solid areas (A): H&E, x200, (B): H&E, x400

    Fig 3: The strong positivity with FVIII-RA revealing endothelial origin of the cells in the solid areas

    Fig 4: The strong positivity with CD34 revealing endothelial origin of the cells in the solid areas

  • Top
  • Introduction
  • Case Presentation
  • Disscussion
  • References
  • Discussion
    The term hemangioendothelioma has been used for designation of vascular tumors with intermediate biologic behavior between hemangioma and angiosarcoma. Although they have the ability for local recurrence, these group tumors have significantly low metastasis risk when compared to angiosarcoma. The epithelioid hemangioendothelioma, the most aggressive type of this family, produces distant metastasis and may result in death. The retiform or Dabska-type hemangioendotheliomas are rarely associated with regional lymph nodes metastasis. The spindle cell hemangioendothelioma has been reclassified by lack of metastasis potential[4,5].

    The previous cases of splenic hemangioendothelioma discussed in the literature have similar histopathological and indolent clinical characteristics similar to the forms described at other sites[6-8]. Commonly, accompanying clinic situation to the splenic hemangioendothelioma is chronic anemia[9]. Functional hyposplenism, spontaneous and traumatic ruptures have been described as single cases[10-12]. The other cases reported in literature were asymptomatic and diagnosed incidentally during abdominal imaging for other reasons. However, our patient presented with right upper quadrant pain due to multiple stones in the gall bladder. A mild anemia and indirect hyperbilirubinemia probably related to splenic hyper function were detected.

    Although spindle cell hemangioendotheliomas are often multicentric when confined to a single anatomic region, the tumors described in the spleen up to date were not multicentric except a case reported by Heese et al[3]. Hemangioendotheliomas are well-separated lesions from healthy parenchyma. In a previously reported case, a primary lesion and multiple intra splenic metastases were described[13]. However, in our case, two distinct tumor masses that do not have continuity with each other were detected. According to our knowledge, this is the second multicentric hemangioendothelioma located in the spleen[3]. Additionally, this is the first case of hemangioendothelioma presenting with indirect hyperbilirubinemia and anemia both of which recovered after splenectomy.

  • Top
  • Introduction
  • Case Presentation
  • Discussion
  • References
  • References

    1) Wick MR, Scheithauer BW, Smith SL, et al. Primary nonlymphoreticular malignant neoplasms of the spleen. Am J Surg Pathol 1982;6:229-42.

    2) Fukunaga M, Ushigome S, Nikaido T, et al. Spindle cell hemangioendothelioma: an immunohistochemical and flow cytometric study of six cases. Pathol Int 1995;45:589-95.

    3) Heese J, Bocklage T. Specimen fine-needle aspiration cytology of littoral cell angioma with histologic and immunohistochemical confirmation. Diagn Cytopathol 2000;22:39-44.

    4) Mentzel T. Hemangioendotheliomas--evolution of a concept of a heterogeneous group of vascular neoplasms. Verh Dtsch Ges Pathol 1998;82:99-111.

    5) Ramer MA, Lumerman H, Kopp W, et al. Epithelioid hemangioendothelioma of the maxilla: case report and review of literature. Periodontal Clin Investig 2001;23:31-5.

    6) Uchimura K, Nakamuta M, Osoegawa M, et al. Hepatic epithelioid hemangioendothelioma. J Clin Gastroenterol 2001;32:431-4.

    7) Pigadas N, Mohamid W, McDermott P. Epithelioid hemangioendothelioma of the parotid salivary gland. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;89:730-8.

    8) Tancredi A, Puca A, Carbone A. Multifocal cerebral hemangio-endothelioma. Case report and review of the literature. Acta Neurochir (Wien) 2000;142:1157-61

    9) Kaw YT, Duwaji MS, Knisley RE, et al. Hemangioendothelioma of the spleen. Arch Pathol Lab Med 1992;116:1079-82.

    10) Budke HL, Breitfeld PP, Neiman RS. Functional hyposplenism due to a primary epithelioid hemangioendothelioma of the spleen. Arch Pathol Lab Med 1995;119:755-7.

    11) Cerda J, Luque Mialdea R, Soleto J, et al. Segmentary splenectomy of the lower tip because of spontaneous rupture of a splenic hemangioendothelioma in a new-born child--a case report. Eur J Pediatr Surg 1994;4:113-5.

    12) Goyal A, Babu SN, Kim V, et al. Hemangioendothelioma of liver and spleen: trauma-induced consumptive coagulopathy. J Pediatr Surg 2002;37:E29.

    13) Tiu CM, Chou YH, Wang HT, et al. Epithelioid hemangioendothelioma of spleen with intrasplenic metastasis: ultrasound and computed-tomography appearance. Comput Med Imaging Graph 1992;16:287-90.

  • Top
  • Introduction
  • Case Presentation
  • Discussion
  • References
  • [ Top ] [ Summary ] [ PDF ] [ Similar Articles ] [ Mail to Editor ]
    Turkish Journal of Cancer web sitesi Novartis Onkoloji'nin karşılıksız eğitim katkılarıyla hazırlanmıştır.
    [ Main Page | Editorial Board | About | Instructions ]
    [ Table of Contents | Archive | Archive Search | Online Submission | E-mail ]