• Users Online: 121
  • Print this page
  • Email this page


 
 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 52  |  Issue : 4  |  Page : 147-150

Retiform and epithelioid hemangioendothelioma arising from the spleen


1 Department of Surgery, Division of Colon and Rectal Surgery, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City; School of Medicine, Tzu Chi University, Hualien, Taiwan
2 Department of Surgery, Division of Colon and Rectal Surgery, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City, Taiwan
3 School of Medicine, Tzu Chi University, Hualien; Department of Surgery, Division of General Surgery, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City, Taiwan

Date of Submission05-Mar-2019
Date of Decision29-Apr-2019
Date of Acceptance25-Jun-2019
Date of Web Publication27-Aug-2019

Correspondence Address:
Dr. Chieh-Wen Lai
Department of Surgery, Division of General Surgery, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, No. 289, Jianguo Road, Xindian City, New Taipei City
Taiwan
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_22_19

Rights and Permissions
  Abstract 


Splenic hemangioendothelioma (HE) is an extremely rare vascular tumor with an uncertain malignant potential. Experience of treatment with this tumor is limited due to their rare occurrence. We herein describe a case of a 53-year-old woman who was found a splenic tumor incidentally. Radiological findings revealed marked splenomegaly with multiple ill-defined hypodense masses as well as a low-density nodule at the left lobe of the liver. The patient underwent splenectomy and wedge resection of the liver. Final pathology confirmed the diagnosis of an admixture of retiform and epithelioid HE of the spleen with metastasis of the liver. However, distant metastases of bilateral lungs developed 6 months after the operation. She received oral thalidomide therapy without significant progression of the disease over a period of 26 months' follow-up. To the best of our knowledge, only 11 cases had been described in the literature. We report the first case of splenic HE metastatic to both liver and lungs. Our patient received thalidomide, a drug with antiangiogenic action, was shown to let the disease progression slow down. It may be recommended for those patients with relapsed disease or metastases.

Keywords: Epithelioid, hemangioendothelioma, retiform, spleen, thalidomide


How to cite this article:
Chen CW, Hsiao KH, Lu TJ, Lai CW. Retiform and epithelioid hemangioendothelioma arising from the spleen. Formos J Surg 2019;52:147-50

How to cite this URL:
Chen CW, Hsiao KH, Lu TJ, Lai CW. Retiform and epithelioid hemangioendothelioma arising from the spleen. Formos J Surg [serial online] 2019 [cited 2019 Sep 21];52:147-50. Available from: http://www.e-fjs.org/text.asp?2019/52/4/147/265491




  Introduction Top


Primary splenic tumors are classified as lymphoid tumors, nonhematolymphoid tumors, and tumor-like lesions. Vascular proliferation accounts for the majority of nonhematolymphoid neoplasms of the spleen. Among these tumors, hemangiomas are the most common benign lesions, and angiosarcomas are the most common malignant ones.[1] Hemangioendotheliomas (HEs) of the spleen are very rare. It is difficult to make a clear differential diagnosis among these neoplasms of the spleen except when tissue proven. A case of splenic HE metastatic to liver and lungs was presented. The literature was also reviewed concerning primary splenic HE.


  Case Report Top


A 53-year-old female was referred to us with a large splenic tumor found incidentally when she received examinations for the impression of systemic lupus erythematosus. Physical examination was unremarkable except for splenomegaly. The hematologic analysis showed normocytic anemia (hemoglobin 11.4 g/dL) and thrombocytopenia (platelet count 69 × 103/μL). Other laboratory parameters were within normal limits. Chest film revealed unremarkable. Ultrasonography of the abdomen showed a marked ill-defined heterogeneous mass of the spleen and small scattered hypoechoic lesions of the liver. The computed tomography (CT) of the abdomen revealed marked splenomegaly with diffuse, multiple ill-defined hypodense masses with heterogeneous contrast medium enhancement. The left lobe of the liver also had hypodense nodules [Figure 1]. Radiologically, splenic tumor with metastasis of the liver was suspected, which malignant lymphoma was a consideration. Ultrasonography-guided needle biopsy of the spleen was performed, and the tissue diagnosis was HE, characterized by abundant vascular channels of various size and focal crowded atypical epithelioid endothelial cells in the stroma.
Figure 1: Computed tomography of the abdomen revealed marked splenomegaly with diffuse, multiple ill-defined low density masses, and heterogeneous contrast medium enhancement of the solid component

Click here to view


At laparotomy, the spleen was nearly totally replaced by the tumor. Some scattered nodules involving the left lobe of the liver were noted. No peritoneal or lymph node involvement was found. The patient underwent splenectomy and partial hepatectomy. Grossly, the spleen weighted 864 g and measured 19 cm × 15 cm × 10 cm. It was well-circumscribed, and nearly entirely replaced by tumor masses [Figure 2]. Microscopically, the spleen specimen showed proliferative spindle-shaped tumor cells with focal hyaline stroma associated with anastomosing vascular channels of variable sizes and congestion. Focal tumor cells in hobnail appearance and intravascular papillations were recognized. There was mild cellular atypia, and the mitotic activity was absent [Figure 3]a, [Figure 3]b, [Figure 3]c Immunohistochemical stains revealed the tumor cells to be focally positive for factor VIII, CD-34, and vimentin, but negative for cytokeratin. A diagnosis of HE, admixture of retiform and epithelioid types was made. The specimen of the liver showed epithelioid HE characterized by proliferation of plump epithelioid and histiocytic tumor cells with cytoplasmic vacuoles in a focal myxoid background and focal spindle cell pattern. Immunohistochemical stains were also positive for vascular markers factor VIII, CD-34, and vimentin [Figure 3]d. The resection margin was free of tumor.
Figure 2: Surgical findings showing the spleen as a well-circumscribed nonencapsulated, and nearly entirely replaced by tumor masses

Click here to view
Figure 3: (a) The specimen of the spleen showed proliferative spindle-shaped and epithelioid tumor cells surrounded by focal hyline stroma (H and E, ×200). (b) Focal tumor cells in hobnail appearance (H and E, ×100). (c) Intravascular papillations were recognized (H and E, ×100). (d) The specimen of the liver showed proliferation of plump epithelioid and histiocytic tumor cells with cytoplasmic vacuoles in a focal myxoid background (H and E, ×200)

Click here to view


The postoperative course was uneventful. No local recurrence was detected, and the hematological tests were normal. Unfortunately, the patient developed distant metastases of bilateral lungs 6 months after the operation. Then she received oral thalidomide therapy (200 mg daily). She was followed by serial CT scans of the abdomen and chest films every 4 months. During 26 months' follow-up, she was asymptomatic and the lung metastases showed no significant progression.


  Discussion Top


Although quite rare, the spectrum of vascular proliferations of the spleen range from benign to malignant, including hemangioma, hamartoma, lymphangioma, HE, hemangiopericytoma, littoral cell angioma, Kaposi's sarcoma, and angiosarcoma. Despite the use of ultrasonography, color Doppler, and CT, it is not easy to make a clear differential diagnosis among these unusual entities. Thus, a needle biopsy or splenectomy is necessary to obtain a definitive tissue diagnosis.

HE is currently synonymous with a morphologically heterogeneous group of vascular neoplasms with low-grade or borderline malignancy, intermediate in both biological behavior and histological appearance between hemangioma and angiosarcoma.[2] The morphological appearance of HEs is highly variable and has been expanded to encompass epithelioid HE, spindle cell HE, retiform HE, kaposiform HE, and polymorphous HE.[3] However, it is difficult to categorize HEs definitively into one of these defined subtypes, since they may sometimes reveal areas of admixture in histological appearance.[4]

Epithelioid HEs are characterized by areas composed of cords of round to spindle-shaped endothelial cells embedded in a myxohyaline stroma. Cytoplasmic vacuoles can be observed in these areas. The tumor had been described to arise in skin, lung, liver, spleen, bone, and brain. There is a high rate of local recurrence and systemic metastases.[5] Retiform HEs arise mainly in the distal extremities. The morphologic features are the presence of long arborizing blood vessels arranged in a retiform pattern lined by monomorphic hobnail endothelial cells, and the focal presence of papilla with hyaline collagenous cores. It is recognized to have low malignant potential, with a high rate of local recurrence but a low frequency of metastasis.

HEs arising from the spleen are extremely rare. To the best of our knowledge, only 11 cases had been described in the literature [Table 1]. Together with our case, there were 8 women and 4 men, with a mean age of 31 years (range, 3–67). These patients were usually found to have a splenic mass incidentally. The most common clinical characteristics are hematological abnormalities, such as anemia and thrombocytopenia. Abdominal pain was the most common presenting symptom. Six of the 12 patients developed distant metastases (50%). The liver is the most common site of metastasis (5 patients), followed by the lung (1 patient) and brain (1 patient). At least three of the twelve patients died of disease after the operation. However, there was no results of long-term follow-up.
Table 1: Summary of the reported cases of splenic hemangioendotheliomas

Click here to view


Imaging findings of splenic HEs are nonspecific. On sonogram and color Doppler imaging, HE is typically seen as a hypoechoic mass with disordered vascularization and high-velocity arterial flow that reflects neoangiogenesis of the tumor. CT scan typically reveals a low-density mass with contrast medium enhancement of the solid portion of the tumor. It is relatively hypovascular compared with the normal splenic parenchyma.[6] Histologically, HEs are composed of vascular and stromal elements with variable morphologic appearance of tumor cells. Immunohistochemical studies show positive staining with antibodies to factor VIII-related antigen, CD-34, and vimentin. HEs are distinguished from hemangioma and angiosarcoma by the features of mild cellular atypia and low mitotic rate.

Hemangiomas, the most common benign neoplasms in the spleen, are usually solitary and incidental findings. These tumors are well circumscribed, measuring 5–8 cm, with an average splenic weight of 300–400 g. Histological features are interconnected, dilated vascular spaces lined by plump, benign-appearing endothelial cells with intervening fibrous tissue. The prognosis is excellent.[7] In contrast, angiosarcomas appear as ill-defined, nodular masses and nearly replace the organ with average splenic weight of more than 1000 g. Histological features are disorganized, anastomosing vascular channels lined by plump, atypical endothelial cells with large, hyperchromatic nuclei and a high mitotic rate. Most patients present with widespread metastases and complicated events such as splenic rupture. They are highly aggressive, with a median survival rate of <1 year.


  Conclusions Top


Our presenting case fully satisfies the definition of HE in both clinical and pathological features. However, the experience of treatment with splenic HE is limited due to their rare occurrence. Surgical extirpation is the mainstay treatment of choice when there is the absence of metastasis. Several antineoplastic agents including thalidomide, cyclophosphamide, adriamycin, and mitomycin had been reported to have varying success in the treatment of hepatic epithelioid HE.[8] Our patient received thalidomide, a drug with antiangiogenic action, was shown to let the disease progression slow down. It may be recommended for those patients with relapsed disease or metastases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgments

This study was supported by the research department of Taipei Tzu Chi hospital, Buddhist Tzu Chi Medical Foundation.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Smith VC, Eisenberg BL, McDonald EC. Primary splenic angiosarcoma. Case report and literature review. Cancer 1985;55:1625-7.  Back to cited text no. 1
    
2.
Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: A vascular tumor often mistaken for a carcinoma. Cancer 1982;50:970-81.  Back to cited text no. 2
    
3.
Nayler SJ, Rubin BP, Calonje E, Chan JK, Fletcher CD. Composite hemangioendothelioma: A complex, low-grade vascular lesion mimicking angiosarcoma. Am J Surg Pathol 2000;24:352-61.  Back to cited text no. 3
    
4.
Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM. Epithelioid hemangioendothelioma and related lesions. Semin Diagn Pathol 1986;3:259-87.  Back to cited text no. 4
    
5.
Silverman ML, Federman M, O'Hara CJ. Malignant hemangioendothelioma of the spleen. A case report with ultrastructural observations. Arch Pathol Lab Med 1981;105:300-4.  Back to cited text no. 5
    
6.
Pines B, Rabinovitch J. Hemangioma of the spleen. Arch Pathol 1942;33:487-503.  Back to cited text no. 6
    
7.
Chen KT, Bolles JC, Gilbert EF. Angiosarcoma of the spleen: A report of two cases and review of the literature. Arch Pathol Lab Med 1979;103:122-4.  Back to cited text no. 7
    
8.
Mascarenhas RC, Sanghvi AN, Friedlander L, Geyer SJ, Beasley HS, Van Thiel DH. Thalidomide inhibits the growth and progression of hepatic epithelioid hemangioendothelioma. Oncology 2004;67:471-5.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
Discussion
Conclusions
References
Article Figures
Article Tables

 Article Access Statistics
    Viewed73    
    Printed2    
    Emailed0    
    PDF Downloaded12    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]