Orbital Haemangiopericytoma

Orbital haemangiopericytoma is a rare soft-tissue neoplasm. This tumour originates from pericytes found around capillaries. Pericyte is a spindle shaped cell with a moderate amount of cytoplasm and an indistinct border. Routine histopathologic differentiation of pericyte from fibroblast, histiocyte and endothelial cell is difficult. Cellular differentiation may be best achieved by electron microscopy and immune-histochemistry.The function of pericytes is uncertain but is believed to provide mechanical support to the capillaries. Pericytes morphologically resemble smooth muscle. Besides the muscle cone of the eye, this tumour may also affect optic nerve meninges and lacrimal sac. This rare tumour is commonly encountered in lower extremities and retro-peritoneum.

Median age of occurrence is fourth decade, but haemangiopericytoma has been noted from 20 months to 87 years of age. The occurrence of tumour in orbit is more common in males.

Orbital haemangiopericytoma has similar signs and symptoms, and imaging study findings as orbital cavernous haemangioma. The tumour generally present with proptosis, diminution of vision, diplopia, and swelling. However, haemangiopericytoma may be more aggressive, extend throughout in orbit, and may even invade the cranial cavity. About one-third of Orbital haemangiopericytoma have histopathologic criteria compatible with malignancy. Distant metastasis of the tumour is uncommon.

 

 

References

Shields Jerry A, Shields Carol L. Eyelid, Conjunctival, and Orbital Tumors- An Atlas and Textbook Second Edition. Lippincott Williams & Wilkins, a Wolters Kluwer business 2008. P 530.

Rootman Jack. Diseases of the Orbit- A Multidisciplinary Approach Second Edition. Lippincott Williams & Wilkins 2003. P 544.

Karcioglu Zeynel A. Orbital Tumors- Diagnosis and Treatment Second Edition. Springer Science+ Business Media, New York 2015. P 168- 171.

Dutton Jonathan J, Gayre Gregg S, Proia Alan D. Diagnostic Atlas of Common Eyelid Diseases. Taylor & Francis Group, LLC 2007. P 159- 161.

Perry Julian D, Singh Arun D. Clinical Ophthalmic Oncology- Orbital Tumors Second Edition. Springer- Verlag Berlin Heidelberg 2014. P 63- 66.

Halperin Edward C, Perez Carlos A, Brady Luther W. Perez and Brady’s Principles and practice of Radiation Oncology Fifth Edition. Lippincott Williams & Wilkins, a Wolters Kluwer business,PA, USA 2008. P 1002- 1005.

Selvakumar Ambika, Noronha Veena, Sundaram Padmaja Minakshi. Sankara Nethralaya Atlas of Imaging in Ophthalmology. Jaypee Brothers Medical Publishers (P) Ltd 2014. P 212- 214.

Eagle Ralph C,Jr. Eye Pathology- An Atlas and Text Second Edition. Lippincott Williams & Wilkins, a Wolters Kluwer business 2011. P 266.

Singh Arun D, Damato Bertil E, Péer Jacob, Murphree A Linn, Perry Julian D. Essentials of Ophthalmic Oncology. Slack Incorporated 2009. P 223.

Black Evan H, Nesi Frank A, Calvano Christopher J, Gladstone Geoffrey J, Levine Mark R. Smith and Nesi’s Ophthalmic Plastic and Reconstructive Surgery Third Edition. Springer Science+Business Media, LLC 2012. P 927- 928.

http://eyewiki.aao.org/Hemangiopericytoma

http://www.scielo.br/pdf/rbcp/v27n3/en_29.pdf

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3862697/

 

Symptoms may include

  • Proptosis (commonly it is painless)
  • Mass effect in the form of swelling predominantly in the superior part of orbit
  • Diminution of vision
  • Diplopia
  • Disturbances in ocular motility

Frequent intracranial extension and involvement of nasal sinuses may produce additional symptoms.

 

There is controversy about the existence of haemangiopericytoma. Its derivation from pericytes is questioned and cases have been reclassified as solitary fibrous tumours, based on histopathologic and immune-histochemical findings. Traditionally, it has been categorised as vascular tumour.

Orbital haemangiopericytoma is believed to originate from extra-vascular pericytes, morphologically resembling smooth muscles around capillaries or from primitive mesenchymal cells. The function of pericyte is uncertain, but is believed to provide mechanical support to the capillaries, and may be having contractile function.

Orbital haemangiopericytoma occurs mainly in adults and has similar symptoms, signs, and imaging studies as cavernous haemangioma. Haemangiopericytoma may be more aggressive with time, extend throughout the orbit, and even invade the cranial cavity.

The main clinical features are proptosis and mass effect due to swelling, predominantly in the superior part of orbit, usually not associated with pain, features of infiltration, or entrapment. Patients usually have symptoms for less than one year on presentation, but there may be variation from one month to twenty six years.

 

Investigations:

  • Ultrasound: Ultrasound shows an encapsulated and well defined solid mass with low internal reflectivity. Orbital haemangiopericytoma may undergo cystic changes within necrotic zones and may resemble the echo-graphic appearance of lymphangioma.
  • Angiography: Angiography shows dilated feeding arteries, early tumour blush, and a rapid venous out flow. Haemangiopericytoma show rapid circulation with significant shunting of blood.
  • CT and MR imaging: On CT and MR imaging, Tumour present as well defined mass with homogeneous enhancement.

 

Histopathology:

Tumour shows uniformly cellular pattern with a sinusoidal vascular component often forming branching (stag horn) channels. Reticulin forms a dense mesh around individual cells. Pericytes show staining for vimentin (protein seen in mesenchymally derived cells) only.

In addition, haemangiopericytoma show myxoid, cellular and cystic component. Giant cells may be seen as well as areas of haemorrhage, necrosis and hyalinisation.

Three patterns have been described:

  • Solid
  • Sinusoidal
  • Mixed

Histopathologically, haemangiopericytoma is divided into three types:

  • Benign tumour
  • Intermediate (Borderline) tumour
  • Malignant tumour

Benign tumour shows minimal atypical cells with few mitotic figures. Intermediate and malignant tumour shows increasing mitosis, pleomorphism, necrosis, haemorrhage, compression of vascular spaces, and infiltrative margins. These tumours also show higher rate of metastasis as compared to benign one. However, distant metastasis is uncommon.

 

Differential diagnosis:

  • Infantile capillary haemangioma
  • Solitary fibrous tumour
  • Mesenchymal chondrosarcoma: Mesenchymal chondrosarcoma show areas of chondroid or cartilaginous tissue.
  • Fibrous histiocytoma: Fibrous histiocytoma show highly cellular pattern without a significant vascular component.
  • Cavernous haemangioma
  • Lymphangioma
  • Meningioma
  • Schwannoma

Optic nerve meningioma and dural sarcoid also needs to be differentiated, in cases where haemangiopericytoma arises from dural sheath.

 

Management of the tumours is mainly surgical.

Surgical therapy

  • Local Excision: Since these tumours usually have a pseudo-capsule, complete local excision is recommended, but it is also difficult because of friability.
  • Exenteration: Tumours with aggressive local behaviour may require exenteration (complete surgical removal of the eyeball and all contents of the eye socket).

Local recurrence may occur with incomplete or piecemeal excision. Recurrence is higher in malignant and borderline types. Even histologically diagnosed tumours may also recur or show distant metastasis. Since local recurrence and metastatic disease is a significant risk in these tumours, long term follow up (at least ten years) is necessary to ensure complete cure.

Orbital recurrence may require wider orbital exenteration, irradiation, or chemotherapy.

Radiotherapy

Radiotherapy is not much of benefit and it may precipitate sarcomatous changes as well.

 

Prognosis

Since even more histologically benign lesions may result in clinically invasive disease or malignant transformation, the aggressiveness of a particular lesion is difficult to predict. Therefore, this tumour warrants aggressive surgery and often adjuvant therapy.

  • PUBLISHED DATE : Jun 20, 2018
  • PUBLISHED BY : NHP Admin
  • CREATED / VALIDATED BY : Dr. S. C. Gupta
  • LAST UPDATED ON : Jun 20, 2018

Discussion

Write your comments

This question is for preventing automated spam submissions
The content on this page has been supervised by the Nodal Officer, Project Director and Assistant Director (Medical) of Centre for Health Informatics. Relevant references are cited on each page.