This document provides an overview of unusual nonepithelial tumors of the head and neck, focusing on glomus tumors. It discusses the anatomy, epidemiology, clinical presentation, diagnosis, classification, and management of glomus tumors. Glomus tumors are rare, usually benign tumors that can develop in various head and neck locations. Surgery is the primary treatment, but radiation therapy may also be used, especially for tumors involving critical structures. Both surgery and radiation have shown success in controlling glomus tumors, though regrowth is still possible.
Treatment Deintensification in HPV positive head and neck cancerDr Rushi Panchal
This ppt is providing detail of current status and future direction of treatment deintensification strategies of head and neck cancer in era of HPV positive sq cell carcinoma.
Conformal Radiotherapy in Head and neck cancers is essential in terms of improving quality of life and local control in this era. This presentation aimed at giving an overview of conformal radiotherapy and its role in HNC to a 'general audience'.
Treatment Deintensification in HPV positive head and neck cancerDr Rushi Panchal
This ppt is providing detail of current status and future direction of treatment deintensification strategies of head and neck cancer in era of HPV positive sq cell carcinoma.
Conformal Radiotherapy in Head and neck cancers is essential in terms of improving quality of life and local control in this era. This presentation aimed at giving an overview of conformal radiotherapy and its role in HNC to a 'general audience'.
Differential diagnosis of head and neck swellings /certified fixed orthodonti...Indian dental academy
The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and offering a wide range of dental certified courses in different formats.
Intramedullary spinal cord tumor is the rare condition demanding high index of suspicion in diagnosis and high yield surgical expertise to produce good outcome.
Highly malignant tumor of mesenchymal origin.Spindle shaped cells that produce osteoid.2nd most common primary malignant bone tumor after MM.Incidence – 1 to 3 per million per year
Treated by chemo,amputation or rotationplasty
Skull Metastasis From Papillary Thyroid Carcinoma : Case Report and Literatur...komalicarol
Although papillary thyroid carcinoma is a relatively common form of malignancy, metastatic spread to the skull
is exceptional. Here, we report a case of papillary thyroid carcinoma revealed by frontal skull metastasis.
Skull Metastasis from Papillary Thyroid Carcinoma: Case Report and Literature...semualkaira
Although papillary thyroid carcinoma is a relatively common form of malignancy, metastatic spread to the skull
is exceptional. Here, we report a case of papillary thyroid carcinoma revealed by frontal skull metastasis.
Locally advanced Ca prostate
Courtesy : NCCN , Perez, Gunderson and Tepper
Brief outline on management
ADT, Radiotherapy, Surgery indications and Standard of care
New Directions in Targeted Therapeutic Approaches for Older Adults With Mantl...i3 Health
i3 Health is pleased to make the speaker slides from this activity available for use as a non-accredited self-study or teaching resource.
This slide deck presented by Dr. Kami Maddocks, Professor-Clinical in the Division of Hematology and
Associate Division Director for Ambulatory Operations
The Ohio State University Comprehensive Cancer Center, will provide insight into new directions in targeted therapeutic approaches for older adults with mantle cell lymphoma.
STATEMENT OF NEED
Mantle cell lymphoma (MCL) is a rare, aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for 5% to 7% of all lymphomas. Its prognosis ranges from indolent disease that does not require treatment for years to very aggressive disease, which is associated with poor survival (Silkenstedt et al, 2021). Typically, MCL is diagnosed at advanced stage and in older patients who cannot tolerate intensive therapy (NCCN, 2022). Although recent advances have slightly increased remission rates, recurrence and relapse remain very common, leading to a median overall survival between 3 and 6 years (LLS, 2021). Though there are several effective options, progress is still needed towards establishing an accepted frontline approach for MCL (Castellino et al, 2022). Treatment selection and management of MCL are complicated by the heterogeneity of prognosis, advanced age and comorbidities of patients, and lack of an established standard approach for treatment, making it vital that clinicians be familiar with the latest research and advances in this area. In this activity chaired by Michael Wang, MD, Professor in the Department of Lymphoma & Myeloma at MD Anderson Cancer Center, expert faculty will discuss prognostic factors informing treatment, the promising results of recent trials in new therapeutic approaches, and the implications of treatment resistance in therapeutic selection for MCL.
Target Audience
Hematology/oncology fellows, attending faculty, and other health care professionals involved in the treatment of patients with mantle cell lymphoma (MCL).
Learning Objectives
1.) Identify clinical and biological prognostic factors that can guide treatment decision making for older adults with MCL
2.) Evaluate emerging data on targeted therapeutic approaches for treatment-naive and relapsed/refractory MCL and their applicability to older adults
3.) Assess mechanisms of resistance to targeted therapies for MCL and their implications for treatment selection
Ozempic: Preoperative Management of Patients on GLP-1 Receptor Agonists Saeid Safari
Preoperative Management of Patients on GLP-1 Receptor Agonists like Ozempic and Semiglutide
ASA GUIDELINE
NYSORA Guideline
2 Case Reports of Gastric Ultrasound
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Explore natural remedies for syphilis treatment in Singapore. Discover alternative therapies, herbal remedies, and lifestyle changes that may complement conventional treatments. Learn about holistic approaches to managing syphilis symptoms and supporting overall health.
Recomendações da OMS sobre cuidados maternos e neonatais para uma experiência pós-natal positiva.
Em consonância com os ODS – Objetivos do Desenvolvimento Sustentável e a Estratégia Global para a Saúde das Mulheres, Crianças e Adolescentes, e aplicando uma abordagem baseada nos direitos humanos, os esforços de cuidados pós-natais devem expandir-se para além da cobertura e da simples sobrevivência, de modo a incluir cuidados de qualidade.
Estas diretrizes visam melhorar a qualidade dos cuidados pós-natais essenciais e de rotina prestados às mulheres e aos recém-nascidos, com o objetivo final de melhorar a saúde e o bem-estar materno e neonatal.
Uma “experiência pós-natal positiva” é um resultado importante para todas as mulheres que dão à luz e para os seus recém-nascidos, estabelecendo as bases para a melhoria da saúde e do bem-estar a curto e longo prazo. Uma experiência pós-natal positiva é definida como aquela em que as mulheres, pessoas que gestam, os recém-nascidos, os casais, os pais, os cuidadores e as famílias recebem informação consistente, garantia e apoio de profissionais de saúde motivados; e onde um sistema de saúde flexível e com recursos reconheça as necessidades das mulheres e dos bebês e respeite o seu contexto cultural.
Estas diretrizes consolidadas apresentam algumas recomendações novas e já bem fundamentadas sobre cuidados pós-natais de rotina para mulheres e neonatos que recebem cuidados no pós-parto em unidades de saúde ou na comunidade, independentemente dos recursos disponíveis.
É fornecido um conjunto abrangente de recomendações para cuidados durante o período puerperal, com ênfase nos cuidados essenciais que todas as mulheres e recém-nascidos devem receber, e com a devida atenção à qualidade dos cuidados; isto é, a entrega e a experiência do cuidado recebido. Estas diretrizes atualizam e ampliam as recomendações da OMS de 2014 sobre cuidados pós-natais da mãe e do recém-nascido e complementam as atuais diretrizes da OMS sobre a gestão de complicações pós-natais.
O estabelecimento da amamentação e o manejo das principais intercorrências é contemplada.
Recomendamos muito.
Vamos discutir essas recomendações no nosso curso de pós-graduação em Aleitamento no Instituto Ciclos.
Esta publicação só está disponível em inglês até o momento.
Prof. Marcus Renato de Carvalho
www.agostodourado.com
Acute scrotum is a general term referring to an emergency condition affecting the contents or the wall of the scrotum.
There are a number of conditions that present acutely, predominantly with pain and/or swelling
A careful and detailed history and examination, and in some cases, investigations allow differentiation between these diagnoses. A prompt diagnosis is essential as the patient may require urgent surgical intervention
Testicular torsion refers to twisting of the spermatic cord, causing ischaemia of the testicle.
Testicular torsion results from inadequate fixation of the testis to the tunica vaginalis producing ischemia from reduced arterial inflow and venous outflow obstruction.
The prevalence of testicular torsion in adult patients hospitalized with acute scrotal pain is approximately 25 to 50 percent
Pulmonary Thromboembolism - etilogy, types, medical- Surgical and nursing man...VarunMahajani
Disruption of blood supply to lung alveoli due to blockage of one or more pulmonary blood vessels is called as Pulmonary thromboembolism. In this presentation we will discuss its causes, types and its management in depth.
Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
These simplified slides by Dr. Sidra Arshad present an overview of the non-respiratory functions of the respiratory tract.
Learning objectives:
1. Enlist the non-respiratory functions of the respiratory tract
2. Briefly explain how these functions are carried out
3. Discuss the significance of dead space
4. Differentiate between minute ventilation and alveolar ventilation
5. Describe the cough and sneeze reflexes
Study Resources:
1. Chapter 39, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 34, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 17, Human Physiology by Lauralee Sherwood, 9th edition
4. Non-respiratory functions of the lungs https://academic.oup.com/bjaed/article/13/3/98/278874
- Video recording of this lecture in English language: https://youtu.be/lK81BzxMqdo
- Video recording of this lecture in Arabic language: https://youtu.be/Ve4P0COk9OI
- Link to download the book free: https://nephrotube.blogspot.com/p/nephrotube-nephrology-books.html
- Link to NephroTube website: www.NephroTube.com
- Link to NephroTube social media accounts: https://nephrotube.blogspot.com/p/join-nephrotube-on-social-media.html
Hemodialysis: Chapter 3, Dialysis Water Unit - Dr.Gawad
Unusual nonepithelial tumors of the head and neck
1. Unusual Nonepithelial Tumors of
the Head and Neck- An overview
Dr Sasikumar Sambasivam
DNB Resident
Dept. of Radiation Oncology
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. 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. • 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. 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. 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. • 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. 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. 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. 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. 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. 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. • 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. 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. • 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. 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. • 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. 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)
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. 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. • 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. • 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. 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%)
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. 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. 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. • 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. 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. 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. 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. 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. 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. • 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. • 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. • 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. • 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. 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