Plan: Radiotherapy (dose 55- 63 Gy) Thats maximum I think we can deliver in this location with photons.

Is there any role for hyperfractionated RT.

What should be the CTV margin/ volume from residual GTV?

Post RT MRI showed no residual disease. Pituitary and hypothalalmus doses were well kept very low. Second MRI last month was also WNL.Clinically he is doing well, with minimal residusal limp. he is under the care of an endocrinologist too.
Any more suggestions for his follow up?

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.

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

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

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.

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

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

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?

vj