Angiosarcoma of the Head, face and Scalp


 

Authors:

Prachi Nayak, M.B.B.S, Postgraduate student

Merline Augustine, M.B.B.S, Postgraduate student

Email:merline13september@gmail.com

Contact no: 8806645530

G. Wiseman Pinto MD, DNB, MNAMS, MIAC

Professor and Head,

Department of Pathology, Goa Medical College, Bambolim, Goa 403202

Email: wisemanpinto@gmail.com

Contact no: 9422641170

 

Abstract

We present here two cases of angiosarcoma of scalp and ear pinna in two elderly men who presented with non healing ulcer.

 

Case Reports:

Case 1:

A 74-year-old male presented with an ulcerated scalp lesion. The patient gave history of trauma followed by scratching which lead to redness and bleeding. The ulcer was excised with a 1cm margin and sent for histopathological examination.

Gross: Specimen of skin with subcutaneous tissue measuring 5 x 4 cms. Skin surface shows a growth measuring 1.5 x 0.8 cms. Cut section revealed a greyish white growth reaching upto the base.

Microscopy: Multiple sections show the dilated vascular channels lined by endothelial cells and composed of fibrous tissue extending upto epidermis. There are areas of haemorrhage, thrombosis, acute on chronic inflammatory infiltrate. Sections from all the margins are free. Sections from the base show presence of haemorrhage. (Figure 1, 2 and 3)

 

TC- Jan 2017 - 005 - Proliferating blood vessels lined by Spindle cells TC- Jan 2017 - 006 - Showing Pleomorphic Spindle cell lining blood vessels

 

TC- Jan 2017 - 007 - Individual Spindle cell

 

Case 2 :

A 67-year-old male presented with left ear pinna non healing ulcer and right upper cervical lymph node enlargement. The ulcer was excised and sent for histopathological examination.

Microscopy: Sections revealed bits of tumour tissue comprising of malignant spindle cells showing moderate amount of cytoplasm, hyperchromatic, pleomorphic nucleus and moderate mitotic activity. At places these cells are seen to line the vascular spaces. Large areas of necrosis are also seen.

Both the cases were concluded as low grade angiosarcoma.

 

Discussion:

The term angiosarcoma refers to a malignant neoplasm arising from the endothelial cells of blood vessels and is therefore synonymous with malignant haemangioendothelioma.

AGE GROUP:

It is usually seen in the adults and elderly but can also occur in the children.

 

SITES:

Most common sites are skin, soft tissue, breast, bone, liver, spleen. Some soft tissue angiosarcoma arise from the major vessels such as inferior vena cava, pulmonary artery or aorta. These tend to have a very undifferentiated appearance and a solid pattern of growth, to such an extent that may not be identifiable as being of endothelial nature.[1] Accordingly topographic terms such as intimal sarcoma, luminal sarcoma, arterial/ venous trunk sarcomas have been used for them.

Also seen in previously irradiated fields, around long standing foreign bodies, in arterio-venous fistulas (including the surgically constructed ones), as a secondary somatic type development in the mediastinal or retroperitoneal germ cell tumors or arising within the pre-existing benign tumors, such as hemangioma/ vascular malformations, neurofibroma, intramuscular lipoma or leiomyoma.

Hepatic angiosarcoma is associated with carcinogenic exposure, including arsenic, thorostat, polyvinyl chloride. All of these agents have long latencies between initial exposure and final tumor development.

Angiosarcoma can also arise in the setting of lymphedema, classically upper extremities several years after radical mastectomy for breast cancer, the tumor presumably arises from the lymphatic vessels (lymphangiosarcoma).

 

GROSS:

Angiosarcoma tend to be highly haemorragic and deeply invasive.

 

MICROSCOPY:

Microscopic appearance ranges from a pattern so well differentiated as to simulate a benign hemangioma to one so undifferentiated and solid as to simulate carcinoma, malignant  melanoma or other types of sarcoma.

The diagnostic areas are represented by freely anastomosing vascular channels lined by atypical endothelial cells, a pattern that is accentuated by silver reticulin stains or immune stains for basement membrane components. Clusters of reactive lymphocytes and clumps of haemosiderin are common. [2]

Variations in appearance of the neoplastic  endothelial cells are great. Their shapes range from very elongated to plump and epitheloid, and their size from small to giant, with occasional development of multinucleated forms. The latter tend to display prominent  hyaline globules containing alpha1 antitrypsin and alpha1 antichymotrypsin.

In rare cases foci of granular cells similar to that seen in granular cell tumors are present. The predominantly epitheloid appearance of the neoplastic endothelial cells can also be seen in the primarily intraluminal tumors of large vessels.

 

DIFFERENTIAL DIAGNOSIS:

  1. Haemangioma – for better differentiated lesions.
  2. Kaposi’s Sarcoma- for those with predominant spindle cell component.
  3. Carcinoma or amelanotic melanoma for poorly differentiated types.
  4. Metastatic Renal Cell Carcinoma – because of its high degree of vascularity, predominantly notorious for its ability to simulate angiosarcoma. In this regard it should be kept in mind that clear tumor cells are not a feature of angiosarcoma.

 

IMMUNOHISTOCHEMISTRY:

Immunohistochemistry and ultrastucturally various endothelial markers can be demonstrated depending upon degree of differentiation. Of these CD31 and FLI-1 are the most reliable. In some cases (particularly epitheloid variant) there is coexpression of keratin. In other cases there is expression of D2-40, suggesting differentiation towards lymph vessel endothelial cells. Ulex Europaes and Vascular Endothelial Growth Factor are also positive.[3]

 

GENES:

Somatic mutation in angiogenesis signalling  genes.

Recurrent mutations in 2 genes, PTPRB(Protein Tyrosine Phosphatase, Receptor Type B) and  PLCG1(Phospholipase C Gamma 1)

 

PROGNOSIS:

Poor prognosis. Recurrence and relapse can  occur.

A well differentiated clinicopathological form of angiosarcoma involves the head and neck region (particularly scalp) of elderly individuals. It begins in the skin but often extends into the subcutis. The clinical course includes repeated  local occurrence over a period of time, followed by in some cases lymph node and pulmonary metastasis.

 

References:

  1. Rich AL, Berman P. Cutaneous angiosarcoma presenting as an unusual facial bruise. Age Ageing. 2004;33:512–4.
  2. Matsumoto K, Inoue K, Fukamizu H, Okayama H, Takigawa M. Prognosis of cutaneous angiosarcoma in Japan: A  statistical study of sixty-nine cases. Chir  Plastica. 1986;8:151.
  3. Lydiatt W M, Shaha A R, Shah J P. Angiosarcoma of the head and neck. Am J Surg. 1994;168(5):451–454.