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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 45-48

Spindle cell hemangioendothelioma presenting as fracture femur – A rare case presentation


1 Department of Pathology, Jawaharlal Nehru Medical College, Faculty of Medicine, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
2 Department of Pathology, Faculty of Dentistry, Jamia Milia Islamia, New Delhi, India
3 Department of Pathology, TMH, Varanasi, Uttar Pradesh, India
4 Department of Pathology, Jawaharlal Nehru Medical College, Faculty of Medicine, Aligarh Muslim University, Aligarh, India

Date of Submission12-Mar-2022
Date of Decision01-Apr-2022
Date of Acceptance05-Apr-2022
Date of Web Publication15-Jun-2022

Correspondence Address:
Dr. Kafil Akhtar
Department of Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aort.aort_6_22

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  Abstract 

Hemangioendothelioma (HE) is a vascular malignancy which shows a borderline biological behavior. Spindle cell HE (SCHE) is a type of hemangioendothelioma, first described by Weiss and Enzinger in 1986. We present a case of 58-year-old male, who presented with fracture right femur. The biopsy showed necrotic bone with features of soft-tissue SCHE. Immunohistochemistry showed diffuse positivity of cluster of differentiation 31 in the tumor cells. Although SCHE has been pondered as a low-grade/borderline malignancy, few cases have been reported as locally aggressive and invasive behavior in the literature. The overall prognosis is excellent and no metastases from this locally aggressive tumor have been reported till date.

Keywords: Hemangioendothelioma, histopathology, immunohistochemistry, pathological fracture


How to cite this article:
Akhtar K, Ara A, Gupta S, Fahim M. Spindle cell hemangioendothelioma presenting as fracture femur – A rare case presentation. Ann Oncol Res Ther 2022;2:45-8

How to cite this URL:
Akhtar K, Ara A, Gupta S, Fahim M. Spindle cell hemangioendothelioma presenting as fracture femur – A rare case presentation. Ann Oncol Res Ther [serial online] 2022 [cited 2022 Jun 26];2:45-8. Available from: http://www.aort.com/text.asp?2022/2/1/45/347561


  Introduction Top


The term hemangioendothelioma (HE) was first coined by Borrmann in 1899.[1] HE is the term that represents various vascular malignancies which show a borderline biological behavior. They are classified as midway between completely benign hemangiomas and extremely malignant angiosarcomas. Spindle cell HE (SCHE) is a type of hemangioendothelioma.

Weiss and Enzinger first explained SCHE in 1986. They defined it as a vascular neoplasm which portrayed the combination of cavernous blood vessels and spindled areas reminiscent of Kaposi's sarcoma.[2] SCHE typically presents as a single tumor or multiple nodules involving the dermis and subcutaneous tissues of the distal extremities.[2] It has a benign indolent course, with a tendency to recur.[3]

We present a case of 58-year-old male, who presented with fracture right femur. The biopsy showed necrotic bone with features of soft-tissue SCHE. Although SCHE has been pondered as a low-grade/borderline malignancy, few cases have been reported as locally aggressive and invasive behavior in the literature. Our case shows the importance of clinicians to be familiar with this rare vascular tumor.


  Case Report Top


A 58-year-old male presented with chief complaints of pain in right lower back and thigh. There was swelling in the right thigh region which was progressive in nature from the past 6 months. There was a history of trauma. There was no history of fever, tuberculosis, or any other chronic illness.

X-ray showed fracture right femur with scalloping cortex and sclerotic changes in femur and nearby soft tissues [Figure 1]. Computed tomography (CT) scan showed an ill-demarcated round mass, with heterogeneous enhancement of the mass and adjacent temporal muscle. A highly vascularized soft-tissue tumor with malignant potential was suspected based on the aggressive features indicated by radiology and rapid growth of the lesion.
Figure 1: X-ray showed fracture right femur with scalloping cortex and sclerotic changes in femur and nearby soft tissues

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An incisional biopsy was performed under general anesthesia to identify the histological variant of malignancy. Histopathological examination showed blood vessels filled partly or completely with erythrocytes or thrombus admixed with cellular zones [Figure 2]. High-power view illustrated blood-filled dilated vessels lined by flattened endothelial cells and spindle-shaped cellular components [Figure 3]. No mitotic figure or nuclear atypia was seen in spindled cells. Immunohistochemistry showed diffuse positivity of cluster of differentiation (CD) 31 in the tumor cells [Figure 4]. A diagnosis of soft-tissue SCHE was given. The patient was administered six cycles of adjuvant chemotherapy (carboplatin, paclitaxel, and bevacizumab in the dosage of 15 mg/kg, every 21 days) and nailing was performed at the fracture site. Our patient is recovering well after 6 months of follow-up period.
Figure 2: Histopathological examination showed blood vessels filled partly or completely with erythrocytes or thrombus admixed with cellular zones. H and E, ×10

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Figure 3: High-power view illustrated blood-filled dilated vessels lined by flattened endothelial cells and spindle-shaped cellular components. No mitotic figure or nuclear atypia was seen in spindled cells. H and E × 40×

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Figure 4: Immunohistochemistry showed diffuse positivity of CD31 in the tumor cells. IHC CD31, ×40. IHC: Immunohistochemistry, CD: Cluster of differentiation

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  Discussion Top


SCHE was first described as a low-grade sarcoma with histologic features intermediate between hemangioma and Kaposi's sarcoma.[4] It is quite difficult to make a specific classification when a given tumor does not hold traits of a one subtype.[4] Recent studies categorize SCHE as a reactive lesion that arises in association with a local congenital or acquired vascular malformation, rather than a neoplastic lesion.[5],[6] There is no demographic prevalence recorded to this rare tumor. The SCHE of long bone-associated soft tissue is very rare. In our case, it was found to be associated with fracture femur in an elderly patient.

The histopathological classification of HEs involving skin and soft tissues includes papillary intralymphatic angioendothelioma (also known as Dabska tumor), retiform HE, kaposiform HE, epithelioid HE, pseudomyogenic HE (also known as epithelioid sarcoma-like HE), SCHE, and composite HE. SCHE is typically described as being composed of two cellular components.[7],[8] In our case, almost similar pattern was observed on histopathology examination, which showed vascular spaces lined by endothelial cells separated by cellular areas of spindle cells, which were distinguished by the focal presence of rounded endothelial cells in nests or lining vascular channels with inflammatory cell infiltration. Abnormal mitotic figures or cellular atypia was not observed.

Subtyping of our patient as SCHE was challenging. Other significant variants of HE such as epithelioid HE and composite HE should be ruled out very clearly by observing each and every focus of the slide.[7] Epithelioid HE is characterized by rounded, eosinophilic epithelioid cells and is composed of cords, strands, and solid aggregates of round, oval, and polygonal cells, with abundant pale eosinophilic cytoplasm, with vesicular nuclei, and inconspicuous nucleoli, embedded in a fibromyxoid or sclerotic stroma. On the other hand, the histologic examination of composite HE shows a vascular tumor with occasional blister cells and a complex combination of histologic patterns consisting of spindle cell hemangioma, retiform HE, and epithelioid HE components.[7],[8]

Immunohistochemical stains of tumor cells are positive for CD31, factor VIII, Ki-67, and vimentin with rare positive foci of D2-40, supporting a neoplasm of vascular origin.[9] The positive CD31, a stain specific for endothelial cells, confirms the presence of endothelial cells.[9],[10] One study showed CD31 positivity with Ki-67 index of 20.0%, consistent with a tumor of intermediate malignant potential. Hisaoka et al. in 12 cases of spindle cell hemangioma reported Ki-67 expression from 0.1% to 14.9%.[11] On immunohistochemistry, the endothelia in the vascular spaces and vacuolated cells show positivity for factor VIII antigen, ulex europaeus agglutinin 1, and vimentin while spindle cells and epithelioid cells react only to vimentin.[10],[11]

Apart from histopathological diagnosis, sometimes, cytopathology is a benchmark tool for recognizing the disease. A cytopathological study by Madhusmita et al. revealed moderate cellularity smear with dissociated single and small clusters of epithelial and spindloid cells in a hemorrhagic background. The cells had round-to-oval vesicular nuclei and some of the cells showed a typical intranuclear pseudoinclusion, with moderate eosinophilic cytoplasm. A cell block preparation on the aspirated material showed vascular spaces and solid areas of spindle and epithelioid endothelial cells with intracytoplasmic vacuoles.[12] Even though we have not performed cytopathology in our case, nevertheless, it is important to say that FNAC should be done wherever possible.

Local recurrence is prevalent in SCHE. Perkins and Weiss reported 58% cases of recurrence at the site of primary growth.[8] The main reason to consider SCHE as a low-grade angiosarcoma is because of such a high recurrence rate.[7] Recent evidences indicate that mostly lesions are partially or purely intravascular.[7],[8] Therefore, “recurrence” is defined as contiguous or multifocal spreading of the lesion along an affected vessel.[8] However, the overall prognosis is excellent and no metastases from SCHE have been reported till date.[8] The reported high rate of local recurrence suggests that excision should extend beyond clinical margins. Our case is doing well, with no complaints of recurrence, after follow-up for 6-month postsurgery.


  Conclusions Top


Due to the paucity of cases of SCHE in literature, the best therapeutic approach and prognosis are still unsolved. Such a rare occurrence of this hardly distinguishable tumor can easily misguide any pathologist and surgeon and one of the most important diagnostic tools is cytological and histological examination of such pathologic lesions.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Weiss SW, Goldblum JR. Hemangioendothelioma: Vascular tumors of intermediate malignancy. In: Strauss M, editor. Enzinger and Weiss's Soft Tissue Tumors. 5th ed. St Louis: Mosby; 2017. p. 891-915.  Back to cited text no. 1
    
2.
Weiss SW, Enzinger FM. Spindle cell hemangioendothelioma. A low-grade angiosarcoma resembling a cavernous hemangioma and Kaposi's sarcoma. Am J Surg Pathol 1986;10:521-30.  Back to cited text no. 2
    
3.
Mahmoudizad R, Samrao A, Bentow JJ, Peng SK, Bhatia N. Composite hemangioendothelioma: An unusual presentation of a rare vascular tumor. Am J Clin Pathol 2014;141:732-6.  Back to cited text no. 3
    
4.
Requena L, Kutzner H. Hemangioendothelioma. Semin Diagn Pathol 2013;30:29-44.  Back to cited text no. 4
    
5.
Meghan F, Daniel M, Donald L. Spindle cell hemangioendothelioma: Natural history over 43 years. Plast Surg Case Stud 2017;3:1-3.  Back to cited text no. 5
    
6.
Das KD, Ghosh K, Kar C, Gharami RC, Chakraborty S, Bhattacharyya A. Spindle cell hemangioendothelioma: Rare clinical entity. Indian J Dermatol 2015;60:509-11.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Suranagi V, Harugop SA, Bannur BH, Pilli SG, Mudhol SR. Spindle cell haemangioma of the nasal cavity: A rare tumor with unusual presentation. Clin Rhinol 2013;6:149-51.  Back to cited text no. 7
    
8.
Perkins P, Weiss SW. Spindle cell hemangioendothelioma. An analysis of 78 cases with reassessment of its pathogenesis and biologic behavior. Am J Surg Pathol 1996;20:1196-204.  Back to cited text no. 8
    
9.
Saikrishna D. Spindle cell haemangioma in head and neck: Report of an uncommon vascular lesion and review of treatment modalities till present. Oral Maxillofac Surg Cases 2020;6:140-9.  Back to cited text no. 9
    
10.
Oukessou Y, Lyoubi M, Hammouda Y, Rouadi S, Abada RL, Roubal M, et al. Spindle cell hemangioma in the infratemporal fossa: A unique case report. Int J Surg Case Rep 2021;78:38-41.  Back to cited text no. 10
    
11.
Hisaoka M, Kouho H, Aoki T, Hashimoto H. DNA flow cytometric and immunohistochemical analysis of proliferative activity in spindle cell haemangioendothelioma. Histopathology 1995;27:451-6.  Back to cited text no. 11
    
12.
Madhusmita J, Suma MN, Padmini J. Spindle-cell hemangioma (spindle-cell hemangioendothelioma): A diagnosis on fine-needle aspiration cytology. Diagn Cytopathol 2012;40:21-5.  Back to cited text no. 12
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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