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Unusual nonepithelial tumors of the head and neck

Unusual nonepithelial tumors of the head and neck

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Unusual nonepithelial tumors of the head and neck

  1. 1. Unusual Nonepithelial Tumors of the Head and Neck- An overview Dr Sasikumar Sambasivam DNB Resident Dept. of Radiation Oncology
  2. 2. 1. Glomus Tumors 2. Hemangiopericytoma 3. Chordomas 4. Lethal Midline Granuloma 5. Chloroma 6. Esthesioneuroblastoma 7. Extramedullary Plasmacytomas 8. Nasopharyngeal Angiofibroma 9. Extracranial Meningiomas 10.Nonlentiginous Melanoma 11.Mucosal Melanomas 12.Lentigo Maligna Melanoma 13.Sarcomas of the Head and Neck
  3. 3. Glomus Tumors •Glomus bodies are found in the jugular bulb and along the tympanic (Jacobson) and auricular (Arnold) branch of the tenth nerve in the middle ear or in other anatomic sites •Depending on the location, glomus tumors (chemodectoma or paraganglioma) can be classified as tympanic (middle ear), jugulare, or carotid vagal, or designated as originating from other locations, such as the larynx, adventitia of thoracic aorta, abdominal aorta, or surface of the lungs
  4. 4. • These tissues are responsive to changes in oxygen and carbon dioxide tensions and pH. • Glomus tumors consist of large epithelioid (smooth muscle) cells with fine granular cytoplasm embedded in a rich capillary network and fibrous stroma with reticulin fibers, which derive from embryonic neural crest cells • Although histologically benign, they may extend along the lumen of the vein to regional lymph nodes, but rarely to distant sites.
  5. 5. Epidemiology •Mean age – 44.7 years for carotid body tumors – 52 years for glomus tympanicum •3 or 4times more frequently in women than in men, suggesting a possible estrogen influence •Glomus tumors may be familial; they also occur in multiple sites in 10% to 20% of patients •Bilateral carotid glomus tumors were reported in six of 16 patients (38%) with a positive family history for these lesions but in only 17/206 patients (8%) without such a history
  6. 6. Clinical Presentation • Glomus tumors of the middle ear may initially cause earache or discomfort • Pulsatile tinnitus • Hearing loss • Cranial nerve paralysis • Middle cranial fossa, symptoms may include temporoparietal headache, retro-orbital pain, proptosis, and paresis of cranial nerves V and VI • Posterior fossa is involved, symptoms may include occipital headache, ataxia, and paresis of cranial nerves V to VII, IX, and XII; invasion of the jugular foramen causes paralysis of nerves IX to XI
  7. 7. • Chemodectoma of the carotid body usually presents as a painless, slowly growing mass in the upper neck. • Mass may be pulsatile and may have an associated thrill or bruit • Mass may extend into the parapharyngeal space and be visible on examination of the oropharynx • Very rarely these tumors may be malignant • Metastases occur in 2% to 5% of cases
  8. 8. Work up History Physical examination ENT examination Basic Investigations Radiographic studies Computed tomography scan (with contrast) to define tumor extent and possible central nervous system involvement Magnetic resonance imaging with gadolinium Arteriography to determine bilateral involvement and collateral cerebral blood flow (optional) Jugular phlebography (optional) Special tests Audiograms to establish baseline hearing loss Histologic staining to determine presence of catecholamines
  9. 9. Glasscock-Jackson Classification of Glomus Tumors Glomus tympanicum I Small mass limited to promontory II Tumor completely filling middle ear space III Tumor filling middle ear and extending into the mastoid IV Tumor filling middle ear, extending into the mastoid or through tympanic membrane to fill the external auditory canal; may extend anterior to carotid Glomus jugulare I Small tumor involving jugular bulb, middle ear, and mastoid II Tumor extending under internal auditory canal; may have intracranial canal extension III Tumor extending into petrous apex; may have intracranial canal extension IV Tumor extending beyond petrous apex into clivus or infratemporal fossa; may have intracranial canal extension
  10. 10. Modification of McCabe and Fletcher Classification of Chemodectomas Group I: Tympanic tumors Absence of bone destruction on x-rays of the mastoid bone and jugular fossa Absence of facial nerve weakness Intact eighth nerve with conductive deafness only Intact jugular foramen nerves (cranial nerves IX, X, and XI) Group II: Tympanomastoid tumors X-ray evidence of bone destruction confined to the mastoid bone and not involving the petrous bone Normal or paretic seventh nerve Intact jugular foramen nerves No evidence of involvement of the superior bulb of the jugular vein on retrograde venogram Group III: Petrosal and extrapetrosal tumors Destruction of the petrous bone, jugular fossa, and/or occipital bone on x-rays Positive findings on retrograde jugulography Evidence of destruction of the petrous or occipital bones on carotid arteriogram Jugular foramen syndrome (paresis of cranial nerves IX, X, or XI) Presence of metastasis
  11. 11. Modification of McCabe and Fletcher Classification of Chemodectomas Group I: Tympanic tumors Absence of bone destruction on x-rays of the mastoid bone and jugular fossa Absence of facial nerve weakness Intact eighth nerve with conductive deafness only Intact jugular foramen nerves (cranial nerves IX, X, and XI) Group II: Tympanomastoid tumors X-ray evidence of bone destruction confined to the mastoid bone and not involving the petrous bone Normal or paretic seventh nerve Intact jugular foramen nerves No evidence of involvement of the superior bulb of the jugular vein on retrograde venogram Group III: Petrosal and extrapetrosal tumors Destruction of the petrous bone, jugular fossa, and/or occipital bone on x-rays Positive findings on retrograde jugulography Evidence of destruction of the petrous or occipital bones on carotid arteriogram Jugular foramen syndrome (paresis of cranial nerves IX, X, or XI) Presence of metastasis
  12. 12. Management • Surgery is generally selected for treatment of small tumors • Glomus tympanicum tumors- excision, via tympanotomy or mastoidectomy • Percutaneous embolization of a low-viscosity silicone polymer has been used, frequently as preoperative preparation of the tumor. Embolization of feeding vessels allows meticulous microsurgery with virtually complete hemostasis • Surgical treatment of a glomus tumor arising in the jugular bulb- piece-by-piece removal • Preoperative embolization via a transarterial approach has proved beneficial but is often limited by vascular anatomy and unfavorable locations
  13. 13. • The local tumor control rate with surgery alone is only about 60%, and there is significant morbidity, particularly cranial nerve injury and bleeding. • In a retrospective review of all skull-base surgery cases treated at Baylor University 175 jugulotympanic glomus tumors and nine malignant cases (5.1%) were identified. The 5year survival rate was 72%.
  14. 14. Radiation Therapy • Tumors with destruction of the petrous bone, jugular fossa, or occipital bone or patients with jugular foramen syndrome are more reliably managed with irradiation. • Some surgeons, such as Glasscock et al. and Spector et al., have questioned the effectiveness of radiation therapy in the treatment of chemodectomas because on histologic sections, obtained even many years after irradiation, it is possible to find chromophilic cells remaining in the tumor. However, there is also evidence of fibrosis and decreased vascularity
  15. 15. • Suit and Gallager demonstrated in a murine mammary carcinoma model that morphologically intact cells may have lost their reproductive ability after irradiation, which is the ultimate end point of cell killing • Extremely unusual to observe clinical regrowth of a glomus tumor after irradiation, even if they do not regress completely. • Some reports describe successful combinations of surgery with either preoperative or postoperative irradiation or preoperatively in an attempt to make an unresectable tumor operable, postoperatively when obvious tumor could not be resected.
  16. 16. Radiation Therapy Techniques • Radiation therapy techniques are determined by the location and extent of the tumor • Limited, usually bilateral, portals should be used for relatively localized glomus tumors • Electrons (15 to 18 MeV) with a lateral portal or combined with cobalt-60 (60Co) or 4- to 6-MV photons (20% to 25% of total tumor dose) render a good dose distribution
  17. 17. • In patients in whom tumor has spread into the posterior fossa, it may be necessary to use parallel opposed portals with 6- to 18-MV photon • Treatment is given at the rate of 1.8 to 2 Gy tumor dose per day with five treatments per week for a total tumor dose of 45 to 55 Gy in 5 weeks. Three-dimention (3D) conformal radiotherapy (RT) or image-guided radiation therapy (IMRT) are highly desirable techniques to treat these tumors, with excellent dose distributions
  18. 18. View Figure Portal used for relatively localized glomus tumor. B: Simulation film of patient with glomus tumor. C: Isodose distribution of a mixed-beam unilateral portal for a glomus tympanicum lesion (80% 16-MeV electrons, 20% 4MV photons)
  19. 19. View Figure Isodose distributions using superiorinferior pairs of 60-degree (A) and 45degree (B) converging wedge filtered 60 Co fields, demonstrating limited volume of irradiation
  20. 20. • Leber et al. reported on 13 patients with glomus tumors treated with radiosurgery because of recurrences after surgical removal in six patients. • Two patients had partial embolization before Gamma Knife (Elekta, Norcross, GA) treatment • Mean follow-up was 42 months (range, 14 to 72 months). • Within the follow-up period there was no tumor progression and no clinical deterioration in any patient • 64% of the patients had an improvement of their symptoms, and in 36% the volume of the lesion decreased in size • There was no radiation-related morbidity
  21. 21. Results of Therapy • • • • Postirradiation change in tumor size is slow Increase in proliferative and perivascular fibrosis Minimal alterations in the chief epithelial cells Histologic evaluation of tumor cell viability is not reliable • Despite the persistence of tumor both clinically and angiographically, amelioration of symptoms, absence of disease progression, and occasional return of cranial nerve function have been reported
  22. 22. • Seventeen patients were treated for glomus tympanicum tumors at Washington University • In five patients initial treatment consisted of irradiation alone, and all were tumorfree at last follow-up (4.5 years in one patient) or at death • Seven of eight patients irradiated for surgical recurrence were free of disease 4.5 to 19 years after irradiation • The remaining four patients were treated preoperatively or postoperatively; only one had recurrence and was salvaged surgically and tumorfree 10 years later • Of six patients with glomus jugulare lesions treated with irradiation, two with extensive lesions died of their disease, whereas the glomus tumor was controlled in four, including two patients with intracranial extension. Irradiation doses ranged from 46 to 52 Gy, with 86% to 100% tumor control with doses over 46 Gy and 50% (two of four) with doses below 46 Gy.
  23. 23. • Of 19 patients treated with irradiation at the M.D. Anderson Cancer Center, five had only a biopsy without any surgical excision and 14 had partial excision, Ten patients had bony destruction; five of these had petrous pyramid and jugular foramen destruction, with accompanying multiple cranial nerve paralysis. Seventeen patients were treated with 60Co anterior-posterior or superior-inferior wedged filtered fields, and two patients received electrons and photons (3:1) via a single lateral field. Of 18 patients surviving a minimum of 5 years (13 surviving more than 10 years), all are alive and free of disease or have died of other causes.
  24. 24. Local Control with Radiation Therapy for Chemodectoma of the Temporal Bone (Glomus Tympanicum and Jugulare) Institution (Reference) Local Control Princess Margaret Hospital (53) 42/45a Nominal Dosage Schedule 35 Gy/3 wk Queen Elizabeth Hospital, Birmingham (6) 45–50 Gy/4–5 wk 19/20b University of Washington (240 10/13 ) 8–65 Gy/4–7 wk Rotterdamsch RadioTherapeutisch Instituut, Netherlands (166) 40–60 Gy/4–6 wk 19/19 University of Minnesota (172) 13/14 30–60 Gy/3.5–7.5 wk University of Virginia (104) 14/17 40–50 Gy/4–5 wk University of Michigan 11/11 Total 128/139 (92%)
  25. 25. Chordomas
  26. 26. Anatomy • Chordomas are rare neoplasms of the axial skeleton that arise from the remnant of the primitive notochord (chorda dorsalis) • About 50% arise in the sacrococcygeal area; 35% arise intracranially, where they typically involve the clivus, and the remaining 15% occur in the midline along the path of the notochord, primarily involving the cervical vertebrae
  27. 27. Epidemiology • Chordomas are more common in patients in their 50s and 60s but can occur in all age groups • In children and young adults the prognosis and long-term survival appear to be better than in older patients • No risk factors have been identified • Male predominance is reported at a 2:1 to 3:1 ratio
  28. 28. Natural History • Slowly growing, chordomas are locally invasive, destroying bone and infiltrating soft tissues • Basisphenoidal chordomas tend to cause symptoms earlier and may be difficult to differentiate histologically from chondromas and chondrosarcomas and radiographically from craniopharyngiomas, pineal tumors, and hypophyseal and pontine glioma • Lethality of these tumors rests on their critical location, aggressive local behavior, and extremely high local recurrence rate
  29. 29. • Incidence of metastasis, which has been reported to be as high as 25%, is higher than previously believed and may be related to the long clinical history • Most common site of distant metastasis is the lungs, followed by liver and bone • Lymphatic spread is uncommon.
  30. 30. Pathology • Chordoma is a soft, lobulated tumor that may have areas of hemorrhage, cystic changes, or calcification • Frequently encapsulated but may be nonencapsulated or pseudoencapsulated • Histologically, it is composed of cords or masses of large cells (physaliferous cells) with typical vacuoles and granules of glycogen in the cytoplasm and abundant intercellular mucoid material • Usually there are few mitotic cells • Heffelfinger et al. postulated that a chondroid variant of chordoma may exist, being prevalent in the spheno-occipital area • Patients with this type of histologic variant have improved survival • Aside from the previously mentioned histologic features, the prognostic factors that most influence the choice of treatment are location and local extent of tumor.
  31. 31. Clinical Presentation • Clinical symptoms vary with the location and extent of the tumor • In the head, extension may be intracranial or extracranial, into the sphenoid sinus, nasopharynx, clivus, and sellar and parasellar areas, with a resultant mass effect • In chordomas of the spheno-occipital region, the most common presenting symptom is headache • Other presentations include symptoms of pituitary insufficiency, nasal stuffiness, bitemporal hemianopsia, diplopia, and other cranial nerve deficits • Fuller and Bloom reported on 13 patients with clivus chordoma, all of whom had multiple cranial nerve palsies. Facial pain was present in 11/13 patients.
  32. 32. Diagnostic Work-up for Chordoma General   History   Physical examination Radiologic studies   Plain radiographs   Computed tomography scan/magnetic resonance imaging Laboratory studies   Complete blood cell count   Chemistry   Urinalysis Special studies   Endocrinologic profile (clivus)   Visual evaluations (clivus)
  33. 33. General Management • Because of their surgical inaccessibility and relative resistance to radiation therapy, clivus chordomas represent a formidable therapeutic challenge. • The general management of the patient is dictated by the anatomic location of the tumor and the direction and extent of spread • A surgical approach is recommended (when feasible), but complete surgical extirpation alone is unusual • Regression of preoperative symptoms without additional postoperative morbidity could be achieved by radical transoral tumor extirpation documented by MRI • Intracranial spread usually requires steroid coverage and therapy directed to correction of neurologic deficits that may be present • Because of the high incidence of local recurrence, combined surgical excision and irradiation is frequently used • No effective chemotherapeutic agent or combination of drugs has been identified.
  34. 34. Radiation Therapy Techniques • Depending on the location of the tumor along the craniospinal axis • Basisphenoidal tumors usually are treated by a combination of parallel opposed lateral fields, anterior wedges, and photon and electron beam combinations • Precision radiation therapy planning, using CT and MRI, is required because high doses of external-beam radiation therapy are needed • Three-dimensional CRT or IMRT provide optimal dose distributions
  35. 35. • Because of the slow proliferative nature of chordomas, high linear energy transfer may prove useful in their management • Brachytherapy can be used for recurrent tumors of the base of skull or adjacent to the spine when a more aggressive surgical exposure is offered. • Three of five chordomas were rendered stable when treated with iodine-94 (94I) implants by Gutin et al., performed with CT stereotactic technique. Kumar et al. reported use of 94I intraoperative interstitial implantation in two patients with recurrent chordomas. • Disease was effectively controlled in both.
  36. 36. • Survival in some patients with chordoma may be long term, the salient feature of this unusual neoplasm is local recurrence with eventual death • Overall 5-year diseasefree survival rate is <10% to 20%. At M.D. Anderson Cancer Center, of 19 patients treated definitively, three were alive and free of disease with relatively short follow-up of 3, 6, and 7.5 years, respectively.
  37. 37. • Catton et al. analyzed the long-term results of treatment for patients with chordoma of the sacrum, base of skull, and mobile spine treated predominantly with postoperative photon irradiation. In 20 base of skull chordomas, most of them irradiated with conventionally fractionated radiation to a median dose of 50 Gy in 25 fractions for 5 weeks (range 25 Gy to 50 Gy), median survival was 62 months (range 4 to 240 months) from diagnosis with no difference between clival and nonclival presentations.
  38. 38. • There was no survival advantage to patients receiving radiation doses >50 Gy (median 60 Gy) compared with lower doses <50 Gy (median 40 Gy). • Hyperfractionation regimens did not influence the degree or duration of symptomatic response or progressionfree survival. • Median survival after retreatment was 18 months.
  39. 39. Protons • The best results in the treatment of chordomas have been obtained with radical surgical procedures followed by high-dose proton irradiation • Tatsuzaki and Urie (261) described the use of proton beam therapy at high doses for chordomas and chondrosarcomas of the base of the skull and cervical spine
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Unusual nonepithelial tumors of the head and neck

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