Case Study 15.1 A 45-year-old woman with a long history of hyperthyroiditis presents with eyelid retraction, exophthalmos, diplopia, and periorbital edema. In her his-tory of illness, she specifi cally notes that she has had deterioration in her vision over the last 10 days accompanied by diplopia 1 day ago. She has not experienced any loss of color vision or demonstrated corneal involvement, but she has minor visual fi eld defects with blurred vision. She is diagnosed with Graves’ disease and thyroid ophthalmopathy. Q: What is your strategy: observation or treatment?
Ugur Selek , Murat Beyzadeoglu , and Gokhan Ozyigit
A: There is a threat of impending visual loss, although the color vision is protected. She does not seem to require decompressive surgery, but orbital radiotherapy could be recommended in addition to systemic immunosuppression with oral or IV steroids. Level I evidence has not demonstrated improvement with radiotherapy in proptosis, eyelid retrac-tion, or soft tissue changes; however, radiotherapy has been recom-mended for its effi cacy for compressive optic neuropathy, which has not yet been clearly determined [ 1 ] . The current recommended external beam radiotherapy dose is 20 Gy (2 Gy/fraction/d) [ 2– 4 ] .
476 15 Rare Tumors and Benign Diseases
A: Standard treatment is medical management with anticonvulsants such as carbamazepine, phenytoin, gabapentin, and baclofen until failure to the control pain [ 5 ] . When medication is not enough to the control pain, treatment options are surgery, such as microvascular decompression, radiofrequency rhizotomy, glycerol rhizotomy, bal-loon compression, peripheral neurectomy, or radiosurgery (approxi-mately 80 Gy in one session) [ 6– 8 ] . Surgery in general ensures acute pain relief, whereas it may take approximately 4–6 weeks with radio-surgery to achieve a decrease in pain.
Case Study 15.2 A 55-year-old woman has been referred to the hospital with right facial pain and paroxysmal attacks lasting approximately 30 s and affecting the ophthalmic and maxillary branches of the trigeminal nerve. Her examination has revealed no gross trigeminal motor or sensory loss, and her history of illness shows that her pain, which is unilateral and can be triggered by talking a lot, brushing her teeth, and smiling, does not wake her up at night. Her MRI ruled out a cerebellopon-tine tumor. She is diagnosed as having trigeminal neuralgia. Q: What is your treatment recommendation?
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Case Study 15.3 A 38-year-old male with complaints of nonspecifi c headaches for almost 6 months has been evaluated by neurology. His brain MRI has demonstrated a recent bleeding and a dark fl ow void surrounding the nidus of an arterio-venous malformation in the thalamus, which was 1.5 cm in size. The angiog-raphy confi rmed this diagnosis in addition to showing no deep venous drainage. He currently has no neurological symptoms. Q: What is his Spetzler-Martin grading?
Q: What is the rough risk of bleeding in his lifetime?
Q: Would you recommend treatment? What is your fi rst option?
A: Size (0–3 cm = 1, 3.1–6.0 cm = 2, >6 cm = 3) + location (nonelo-quent = 1, eloquent = 2) + deep venous drainage (absent = 0, pres-ent = 1) = grade 3 is Spetzler-Martin grading [ 9 ]
A: Lifetime risk of hemorrhage [ 10 ] = 105 – patient age in years = 105 − 38 = 67%
A: This patient has a high lifetime bleeding risk; therefore, treatment needs to be recommended for this young patient. Lesions in eloquent brain areas are very diffi cult to resected safely [ 11 ] . Radiosurgery could be a better option for hemorrhage risk reduction [ 12 ] . Obliteration can vary depending on the dose and the size of the lesion; increasing doses step-wise increases the obliteration rate (16 Gy = 70%; 18 Gy = 80%; 20 Gy = 90%) [ 13– 15 ] . Endovascular embolization could also be a use-ful adjunct to radiosurgery [ 16, 17 ] . A general review is provided by the American Stroke Association publication on recommendations for the management of arteriovenous malformations [ 18, 19 ] .
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Case Study 15.4 A 45-year-old male presents for a hip reconstruction operation after a blunt trauma to his femoral prosthesis. In his workup, radiology demonstrated bone spurs from the proximal femur in addition to a ³ 1-cm gap between the oppos-ing surfaces. The surgeon asks your opinion about prophylaxis of heterotopic ossifi cation in order to choose between a medical or radiotherapy approach. Q: What would you recommend as a prophylaxis?
Q: If you recommend radiotherapy, what is the timing and dose?
A: Radiotherapy is a better option according to the results of a meta- analysis analyzing 7 randomized trials and 1,143 patients [ 20 ] . Radiotherapy is approximately twice as effective as non-steroidal anti-infl ammatory medi-cations. However, the gain in absolute benefi t is shown to be small (<2%).
A: The timing might be preoperative, such as less than 4 h before surgery, and in the early 72 h postoperatively [ 21 ] . The recommended dose is 6 or 7 Gy in one fraction [ 20, 21 ] .
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Q: What is your radiotherapy recommendation if the patient elects to be treated with external beam radiotherapy?
A: A recent metaanalysis demonstrated a better decrease in recurrence rates when the biological effective dose is greater than 30 Gy, which results in a recurrence rate of less than 10% [ 22 ] . Therefore, 18 Gy in three fractions or 12 Gy in one fraction can be recommended within 2 days of surgery [ 22 ] .
Case Study 15.5 A 42-year-old female has been sent to radiation oncology to be evaluated for keloid prophylaxis following planned forearm surgery because of her keloid scar experience after a cesarean section. Q: What is the current standard for her prophylaxis?
A: There is currently no real standard, and a wide range of treatment modalities, such as radiotherapy, cryotherapy, intralesional injections of corticosteroids, surgery, pressure therapy, interferon, fl uorouracil, topi-cal silicone, and pulse-dye laser treatment. However, experience with postoperative radiotherapy is well documented [ 22, 23 ] .
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Case Study 15.6 A 66-year-old male is admitted to the hospital with minimal pain in the bilat-eral breasts, which have become prominent over the last 10 days. He has been using androgen deprivation therapy and a single agent androgen receptor blocker, bicalutamide 150 mg/day, for his diagnosis of metastatic prostate cancer. His examination revealed breast tenderness in deep palpation, but the ultrasound imaging noted no appearance of breast tissue enlargement. Q: To what is this tenderness mostly related?
Q: What is your treatment recommendation? What dose should be used?
A: This clinical appearance seems to indicate the initiation of gyneco-mastia related with androgen deprivation, which is not infrequent in metastatic prostate adenocancer patients undergoing hormone therapy.
A: Antiestrogen treatment with tamoxifen appears to be superior to radiotherapy according to comparative studies [ 24– 26 ] . Tamoxifen shows a dose-dependent response, and 20 mg QD seems to ensure a better outcome. If the patient elects to be treated with radiotherapy, which is also an effective modality, 6–12 MeV electrons with a 5-cm diameter of tissue around each nipple can be delivered to 90% [ 26 ] .
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Case Study 15.7 A 36-year-old male has been sent from the urology department. His status is after a failed minimal surgical procedure for Peyronie’s disease. He has a penis deviation of 40° with minimal pain during erection, which precludes normal coitus. He rejects any further surgical recommendations. Q: Could radiotherapy improve the deviation and relieve the pain?
A: Yes. Recent studies have revealed that radiotherapy is an effective modality to improve deviation (~47%) and relieve pain (~50%) [ 27– 32 ] . Therefore, external beam radiotherapy of 30–36 Gy at 2 Gy/fraction/day can be recommended.
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Case Study 15.8 A 69-year-old female is admitted to radiation oncology with refractory pain because of a dorsal heel spur formed in the Achilles tendon. She complains of severe pain under the heel, causing gait impairment. Her history of illness displays failure of NSAIDS, local anesthesia applications, corticoid suspen-sions, iontophoresis, and microwave/ultrasound procedures. Q: Can radiotherapy reduce this dorsal heel spur pain? What is your prescription?
A: Yes. Radiotherapy is a nonsurgical alternative for patients with pain-ful heel spurs. The neurolysis of the suralis and posterior tibial nerve achieved by radiation results in partial to complete pain relief. External beam radiotherapy of 3 Gy at 0.5-Gy/fraction twice weekly or 8 Gy total irradiation in two fractions is an effective dose to relieve pain in these patients and is preferred for refractory cases [ 33– 35 ] .
48315 Rare Tumors and Benign Diseases
Case Study 15.9 A 59-year-old male has been evaluated by the ear, nose, and throat surgeon because of a recently developed, very mild swallowing defi ciency, and a long history of tinnitus. MRI imaging revealed a 4-cm mass in the middle ear extending and compressing the jugular foramen and cranial nerves. The tumor extendings under the internal auditory canal with an intracranial canal exten-sion of more than 2 cm. His audiological examination revealed a 30% hearing loss. He was diagnosed as having glomus jugulare. Q: What is your treatment recommendation?
A: Although both surgery and radiotherapy (fractionated or radiosur-gery) can be recommended, surgery carries a high morbidity risk in patients because of the above-mentioned extensions [ 36 ] . Therefore, fractionated radiotherapy with doses of 45–55 Gy can be the fi rst option for a lower morbidity approach. Radiosurgery is not a very appropriate option in this case with a tumor compressing the jugular foramen and extending intracranially more than 2 cm. Surgery could be reserved as a salvage treatment [ 36– 41 ] .
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References
1. Bradley EA, Gower EW, Bradley DJ, Meyer DR, Cahill KV, Custer PL et al (2008) Orbital radiation for graves ophthalmopathy: a report by the American Academy of Ophthalmology. Ophthalmology 115(2):398–409
2. Prummel MF, Mourits MP, Blank L, Berghout A, Koornneef L, Wiersinga WM (1993) Randomized double-blind trial of prednisone versus radiotherapy in Graves’ ophthalmopathy. Lancet 342(8877):949–954
3. Prummel MF, Terwee CB, Gerding MN, Baldeschi L, Mourits MP, Blank L et al (2004) A randomized controlled trial of orbital radiotherapy versus sham irradiation in patients with mild Graves’ ophthalmopathy. J Clin Endocrinol Metab 89(1):15–20
4. Wakelkamp IM, Tan H, Saeed P, Schlingemann RO, Verbraak FD, Blank LE et al (2004) Orbital irradiation for Graves’ ophthalmopathy: is it safe? A long-term follow-up study. Ophthalmology 111(8):1557–1562
5. Chole R, Patil R, Degwekar SS, Bhowate RR (2007) Drug treatment of trigeminal neuralgia: a systematic review of the literature. J Oral Maxillofac Surg 65(1):40–45
6. Spatz AL, Zakrzewska JM, Kay EJ (2007) Decision analysis of medical and surgical treat-ments for trigeminal neuralgia: how patient evaluations of benefi ts and risks affect the utility of treatment decisions. Pain 131(3):302–310
7. Brisman R (2007) Microvascular decompression vs. gamma knife radiosurgery for typical trigeminal neuralgia: preliminary fi ndings. Stereotact Funct Neurosurg 85(2–3):94–98
8. Cheng JS, Sanchez-Mejia RO, Limbo M, Ward MM, Barbaro NM (2005) Management of medically refractory trigeminal neuralgia in patients with multiple sclerosis. Neurosurg Focus 18(5):e13
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10. Brown RD Jr (2000) Simple risk predictions for arteriovenous malformation hemorrhage. Neurosurgery 46(4):1024
Case Study 15.10 A 15-year-old male presents with radiological fi ndings of a cavernous angioma with a heterogeneous-appearing 4 × 3-cm right frontal cavernous angioma. The CT scan defi ned central hyperattenuation and a diffuse peripheral area associated with calcifi cations and small areas of hemorrhage. His history of illness includes frequent seizures. Q: What is your treatment recommendation in this case? Could radio-therapy or radiosurgery be the fi rst option?
A: A symptomatic patient with seizures requires surgical intervention. Radiotherapy or radiosurgery is not the fi rst treatment option in the pedi-atric age group or in symptomatic cases [ 42 ] ; brainstem or diencephalic cavernous malformations can be treated with radiosurgery [ 43 ] .
485References
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12. Maruyama K, Kawahara N, Shin M, Tago M, Kishimoto J, Kurita H et al (2005) The risk of hemorrhage after radiosurgery for cerebral arteriovenous malformations. N Engl J Med 352(2):146–153
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14. Flickinger JC, Pollock BE, Kondziolka D, Lunsford LD (1996) A dose–response analysis of arteriovenous malformation obliteration after radiosurgery. Int J Radiat Oncol Biol Phys 36(4):873–879
15. Karlsson B, Lindquist C, Steiner L (1997) Prediction of obliteration after gamma knife surgery for cerebral arteriovenous malformations. Neurosurgery 40(3):425–430; discussion 430–431
16. Hartmann A, Pile-Spellman J, Stapf C, Sciacca RR, Faulstich A, Mohr JP et al (2002) Risk of endovascular treatment of brain arteriovenous malformations. Stroke 33(7):1816–1820
17. Starke RM, Komotar RJ, Otten ML, Hahn DK, Fischer LE, Hwang BY et al (2009) Adjuvant embolization with N-butyl cyanoacrylate in the treatment of cerebral arteriovenous malforma-tions: outcomes, complications, and predictors of neurologic defi cits. Stroke 40(8):2783–2790
18. Ogilvy CS, Stieg PE, Awad I, Brown RD Jr, Kondziolka D, Rosenwasser R et al (2001) AHA Scientifi c Statement: recommendations for the management of intracranial arteriovenous malformations: a statement for healthcare professionals from a special writing group of the Stroke Council, American Stroke Association. Stroke 32(6):1458–1471
19. Ogilvy CS, Stieg PE, Awad I, Brown RD Jr, Kondziolka D, Rosenwasser R et al (2001) Recommendations for the management of intracranial arteriovenous malformations: a state-ment for healthcare professionals from a special writing group of the Stroke Council, American Stroke Association. Circulation 103(21):2644–2657
20. Pakos EE, Ioannidis JP (2004) Radiotherapy vs. nonsteroidal anti-infl ammatory drugs for the prevention of heterotopic ossifi cation after major hip procedures: a meta-analysis of random-ized trials. Int J Radiat Oncol Biol Phys 60(3):888–895
21. Seegenschmiedt MH, Makoski HB, Micke O (2001) Radiation prophylaxis for heterotopic ossifi cation about the hip joint – a multicenter study. Int J Radiat Oncol Biol Phys 51(3):756–765
22. Kal HB, Veen RE (2005) Biologically effective doses of postoperative radiotherapy in the prevention of keloids. Dose-effect relationship. Strahlenther Onkol 181(11):717–723
23. Flickinger JC (2011) A radiobiological analysis of multicenter data for postoperative keloid radiotherapy. Int J Radiat Oncol Biol Phys 79(4):1164–1170
24. Di Lorenzo G, Autorino R, Perdona S, De Placido S (2005) Management of gynaecomastia in patients with prostate cancer: a systematic review. Lancet Oncol 6(12):972–979
25. Di Lorenzo G, Perdona S, De Placido S, D’Armiento M, Gallo A, Damiano R et al (2005) Gynecomastia and breast pain induced by adjuvant therapy with bicalutamide after radical prostatectomy in patients with prostate cancer: the role of tamoxifen and radiotherapy. J Urol 174(6):2197–2203
26. Perdona S, Autorino R, De Placido S, D’Armiento M, Gallo A, Damiano R et al (2005) Effi cacy of tamoxifen and radiotherapy for prevention and treatment of gynaecomastia and breast pain caused by bicalutamide in prostate cancer: a randomised controlled trial. Lancet Oncol 6(5):295–300
27. El-Sakka AI, Yassin AA (2008) Conservative treatment for Peyronie’s disease: an update. Expert Opin Pharmacother 9(1):53–63
28. Incrocci L, Hop WC, Seegenschmiedt HM (2008) Radiotherapy for Peyronie’s disease: a European survey. Acta Oncol 47(6):1110–1112
29. Incrocci L, Hop WC, Slob AK (2000) Current sexual functioning in 106 patients with Peyronie’s disease treated with radiotherapy 9 years earlier. Urology 56(6):1030–1034
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30. Incrocci L, Wijnmaalen A, Slob AK, Hop WC, Levendag PC (2000) Low-dose radiotherapy in 179 patients with Peyronie’s disease: treatment outcome and current sexual functioning. Int J Radiat Oncol Biol Phys 47(5):1353–1356
31. Niewald M, Wenzlawowicz KV, Fleckenstein J, Wisser L, Derouet H, Rube C (2006) Results of radiotherapy for Peyronie’s disease. Int J Radiat Oncol Biol Phys 64(1):258–262
32. Seegenschmiedt MH, Katalinic A, Makoski H, Haase W, Gademann G, Hassenstein E (2000) Radiation therapy for benign diseases: patterns of care study in Germany. Int J Radiat Oncol Biol Phys 47(1):195–202
33. Muecke R, Micke O, Reichl B, Heyder R, Prott FJ, Seegenschmiedt MH et al (2007) Demographic, clinical and treatment related predictors for event-free probability following low-dose radiotherapy for painful heel spurs – a retrospective multicenter study of 502 patients. Acta Oncol 46(2):239–246
34. Heyd R, Tselis N, Ackermann H, Roddiger SJ, Zamboglou N (2007) Radiation therapy for painful heel spurs: results of a prospective randomized study. Strahlenther Onkol 183(1):3–9
35. Surenkok S, Dirican B, Beyzadeoglu M, Oysul K (2006) Heel spur radiotherapy and radiation carcinogenesis risk estimation. Radiat Med 24(8):573–576
36. Kollert M, Minovi A, Mangold R, Hendus J, Draf W, Bockmuhl U (2006) Paraganglioma of the head and neck–tumor control, functional results and quality of life. Laryngorhinootologie 85(9):649–656
37. Semaan MT, Megerian CA (2008) Current assessment and management of glomus tumors. Curr Opin Otolaryngol Head Neck Surg 16(5):420–426
38. Martin JJ, Kondziolka D, Flickinger JC, Mathieu D, Niranjan A, Lunsford LD (2007) Cranial nerve preservation and outcomes after stereotactic radiosurgery for jugular foramen schwan-nomas. Neurosurgery 61(1):76–81; discussion 81
39. Lim M, Bower R, Nangiana JS, Adler JR, Chang SD (2007) Radiosurgery for glomus jugulare tumors. Technol Cancer Res Treat 6(5):419–423
40. Li G, Chang S, Adler JR Jr, Lim M (2007) Irradiation of glomus jugulare tumors: a historical perspective. Neurosurg Focus 23(6):E13
41. Krych AJ, Foote RL, Brown PD, Garces YI, Link MJ (2006) Long-term results of irradiation for paraganglioma. Int J Radiat Oncol Biol Phys 65(4):1063–1066
42. Acciarri N, Galassi E, Giulioni M, Pozzati E, Grasso V, Palandri G et al (2009) Cavernous malformations of the central nervous system in the pediatric age group. Pediatr Neurosurg 45(2):81–104
43. Hasegawa T, McInerney J, Kondziolka D, Lee JY, Flickinger JC, Lunsford LD (2002) Long-term results after stereotactic radiosurgery for patients with cavernous malformations. Neurosurgery 50(6):1190–1197; discussion 7–8
