D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

Dr Nilesh Mahale 10 Mar 2011 12:11

CLINICAL:
PRESENTED \VITH LT. THORACIC RADICULAR PAINS & RECENT ONSET SPASTIC
PARAPARESIS.
MR : IDEM TUMOR AT D 4-5 LEVEL, MAINLY oN LEFT SIDE.

gROSS TOTAL EXCISION DONE

INTRA - OP - RECENT BLEED IN TI{E TUMOR.IT WAS TOUGH, YELLOWISH, WAS ARISING
FROM THORACIC NERVE ROOT & WAS NOT VERY VASCTJLAR
PROVISIONAL DIAGNOSIS: ? NEUROFIBROMA

POST OP IMPROVEMENT IN POWER

GROSS APPEARANCE:
A MASS, I.5 X 0.8 X 0.7 CMS., REDDISH IN COLOUR. C/S SHOWS WHITISH & PINKISH AREAS.
ALL PROCESSED. ( I BLOCK)
HISTOLOGICAL EXAMINATION :
SECTIONS SHOW A GANGLION & A MASS SHOWING AREAS OF HAEMORR.HAGE. CYSTIC
tlEAs ARE sEEN. LoBULAR AREAS WITH MANY SEPERATE CAPILLARIES, sEeERATE PLUMP
CELLS ARE SEEN. NEUCLEI ARE ROUND OR OVAL. MITOSIS IS OCCASIONAL.
FIBROUS AREAS ARE SEEN
IMPRESSION:
D 4-5 IDEM TUMOR - HAEMANGIOPERICYTOMA, WHO GR. II

CAN WE OBSERVE SAFELY?

Re: D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

Santam Chakraborty 10 Mar 2011 15:04

Hi Nilesh what is the age of the patient? and what is your assessment of the extent of resection ?

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Re: D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

Pranshu Mohindra 10 Mar 2011 22:20

Hi Nilesh,

One of those rare tumors where evidence is limited. There have been a few retro. single institution series. I'm sure you already did the pubmed search, but just to list a few:

1 [http://www.ncbi.nlm.nih.gov/pubmed/20672899]

2 [http://www.ncbi.nlm.nih.gov/pubmed/15042684]

3 [http://www.ncbi.nlm.nih.gov/pubmed/12633961]

4 [http://www.ncbi.nlm.nih.gov/pubmed/11322435]

Most of these studies suggest following main points:

1. GTR is best when feasible
2. Adj. EBRT reduces local recurrence significantly (across the board, histology grades not that well documented). But I would imagine, akin to meningioma series, any grade 2 or 3 may merit PORT.
3. Close surveillance is an option especially for low-intermediate grades.
4. Characterised by late recurrences… ten-, fifteen-year follow ups show increased neuraxis and even extra-neural recurrences. It is felt that use of EBRT may not impact this risk.

Without any personal experience whatsoever for this diagnosis, below would be my suggestion based on above studies. Would be nice if others can add their suggestions/ viewpoints for our future reference in similar situations, especially in light of limited evidence.

RT INDICATION:

1. Santam's age question is very valid: Adult patient could possibly be offered EBRT followed by regular (yearly to two yearly neuraxis imaging) for an extended period of time.
2. Pediatric patients: Close imaging surveillance for low-intermediate grade may be considered to delay use of RT.
3. All high-grades and possibly intermediate-grades
4. Sub-total resection

RT DOSE:
I think most series have used 45-60 Gy in neuraxis locations depending on grade and location. In your patient, I think 50.4 Gy in 1.8 Gy/fraction could be safe limiting cord max. point dose to 50.4 Gy. If dosimetry permits, you could aim for 54 Gy as well.

ROLE OF CSI KNOWING RISK OF NEURAXIS RECURRENCES:
I don't think we have any evidence whatsoever on this. I personally would not recommend this.

RT TECHNIQUE:
I guess, one could put a strong point for using highly conformal techniques with image-guidence and good immobilzation to allow adequate dose delivery to target volume and keeping cord dose ALARA. The reason being that in case this unfortunate patient has a late recurrence 5-10 years or more down the line, one could possibly think of coming back with a SRS approach provided cord doses this time have been limited.
Also, it would be a good case to hand-over DICOM image set of radiation plan to patient for his future reference in case he moves out somewhere else, something that most of us under-stress.

Regards

Pranshu

Re: D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

VIMOJ J NAIR 14 Mar 2011 03:10

EBRT 50.4Gy/ 18Fr. - with active physiotherapy.

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But risk of late recurrence. We've seen late extra neural recurrences a decade after the initial treatment.

just for discussion - a differential diagnosis is bcos of similar appearance synovial sarcoma [in other sites] ; ruled out by translocation studies- specific for SS only . when these patients recur after 10-15 years with many mets; some pathologists prefer the diagnosis of SS, and managing with chemo like SS. We have given total metastases irradiation with tomotherapy, but fared poorly.

These were previously diagnosed as angioblastic meningioma. now with all those previously treated cases, recurring systemically, we need to look further deep into the biology of this tumor

http://www.ncbi.nlm.nih.gov/pubmed/21104099

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It is our choices that show what we really are, far more than our abilities.

Re: D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

Nikhilesh Patil 15 Mar 2011 03:02

Hi Nilesh,
We need the details on extent of resection? Post op MRI details ? If in doubt treat with what Pranshu/Vimoj have suggested (~50Gy conventional frac).

One recipe for CNS tumors that I have always remembered.
High Grade lesions: 60Gy/30#'s.
Low Grade: 50-54Gy/1.8 - 2 Gy/#.
Pituitary: 45Gy/1.8 - 2Gy per #.

Consider observation only if you think there was complete excision and you can reliably follow this patient.

Re: D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

Dr Nilesh Mahale 16 Mar 2011 12:12

Thanks for the discussion
Age of this unmarried girl is 2o
Surgeon has said it is gross total excision which I always doubt in Spinal Cord tumours.
Patient has not come to me but known to me So I have advised for post op MR for assessment of residual ds

Re: D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

dr Arun S 16 Mar 2011 16:18

It's nearly impossible to get a complete excision in spinal cord tumors (with clear margins as we would like to have !!) Even if the MR shows no gross residual disease, I would consider RT for her.

Re: D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

Santam Chakraborty 16 Mar 2011 18:47

I would agree with Nilesh's plan and Nikhilesh and Vimoj have given the answer. If nothing the MRI will serve as a good baseline image for follow up purposes. There will never be evidence on this one but if a recurrence is there in this area then the consequences for the patient would be worse. I would give RT and explain to the patient that she has a higher risk than normal of developing a lung / breast cancer in the future. I would also like to treat her as conformal as possible (would be giving fractionated stereotaxy a serious consideration although it can be reserved for a future recurrence too).

1. http://thejns.org/doi/abs/10.3171/jns.1993.78.6.0903?journalCode=jns

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Last edited on 16 Mar 2011 22:46 by Santam Chakraborty Show more

Re: D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

Dr Nilesh Mahale 17 Mar 2011 05:51

Conformal RT is definitely preferable. But is it possible to spare S cord in spinal haemangiopericytoma even with SCRT?

Re: D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

Santam Chakraborty 17 Mar 2011 17:13

Hi Nilesh,
The word sparing is a relative term. As per your description of the IDEM tumor it was situated beside the spinal cord and when you have a concave target volume wrapped around at least half way around the spinal cord (I am sure this is what the target volume would look for this patient after you make the necessary expansions along the meninges) it makes sense the modality that gives the sharpest dose gradient beyond the target will spare the spinal cord to the maximum. Here in is the role of Stereotaxy. You might debate the clinical relevance of this "sparing" though.

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Last edited on 17 Mar 2011 17:15 by Santam Chakraborty Show more

Re: D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

Nikhilesh Patil 18 Mar 2011 02:29

Many companies are selling machines in this area, claiming to be superior in cord sparing, stereotaxy for spinal tumors is big things these days.

Re: D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

radtuxabhishek 20 Mar 2011 05:45

Pranshu has made an excellent and clear cut case for management of Hemangiopericytoma in Spine with Radiation.
Case report and discussion (Different from the abstracts listed above) I'd like to add my observations here.

Although evidence based on the retrospective series cannot be labelled as robust but even for a rare disease, there is a substantial literature. One of the probable reasons is that WHO has re-classified it as non-sarcomatous meningioma which has led to spurt in the "recommendations".

In "Management of Recurrent Meningiomas, authors of clearly made a case for Radiation.

In favor of the Stereotactic Radiation, another article in the same journal (is the largest series of patients treated with stereotactic XRT for hemangiopericytoma), the authors recommend 50+ Gy (related to the tolerance of the structures).

The complete text of the article

Pertinent issues from the main text:

1) All recurrences were localized in-field of the primary RT target volume.

2) For macroscopic residual tumors, the planning target volume (PTV) included the area of contrast enhancement adding a safety margin of 5–20 mm for typical microscopic spread, depending on the location of the tumor and on histology (WHO Grade II vs. WHO Grade III).

3) The most effective drug was shown to be adriamycin, leading to possible complete and partial remission rates of 50%; however, no survival advantage has been seen.

Rest of the issues have already been mentioned above and hence do not merit repetition on my behalf.

I hope this helps

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Reality is merely a persistent illusion

Re: D 4-5 CORD - HAEMANGIOPERICYTOMA, WHO GR. II

VIMOJ J NAIR 20 Mar 2011 16:39

Nice literature search.
I believe there is enough data now to proceed.

Just to add, unlike the other cancers we see, these tumors are seen in relatively younger patients with good KPS till they have delayed extraneuraxial metastasis. So active rehab is needed for a better QOL as they may live longer than malignancies.
But once the mets appear, its rapid deterioration in the cards. We havent seen a good response to chemo. And the above literature complies with that.

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It is our choices that show what we really are, far more than our abilities.