Paraganglioma INTERNET

8/8/2019 Paraganglioma INTERNET

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EXTRA-ADRENAL PARAGANGLIOMA

A brief overview.

Dr. Sampurna Roy M.D.



Paraganglia are specialized neural creast

cells that arise in association with theautonomic ganglia.

The system includes:

Adrenal medulla

Extradrenal paraganglia



EXTRA - ADRENAL

PARAGANGLIOMA- distributionPresent on either side of the midline. Extends

from middle ear region and base of skull to the

pelvic floor.

Classification according to the anatomic sites -

Glenner and Grimley :

1. Branchiomeric (Head and neck) paraganglia2. Intravagal paraganglia

3. Aorticosympathetic paraganglia

4. Visceral autonomic paraganglia



BRANCHIOMERIC PARAGANGLIOMA

Carotid body tumour

Jugulotympanic

Aorticopulmonary Subclavian

Laryngeal

Coronary

Orbital



Paraganglia of the head and neck

(branchiomeric paraganglia)

Closely associated with the parasympathetic

nervous system.

Play an important role as chemoreceptors .

Detect changes in blood pH and oxygen tension



CAROTID BODY

Located on the medial side of the carotid bifurcation on

both side of the neck. Average combined weight is about 12mg

Composed of chief cells and sustentacular cells.

Chief cells are demonstrated by Chromogranin A.

Sustenticular cells are demonstrated by S100 protein.

CAROTID BODY HYPERPLASIA

High altitude, chronic lung disease (emphysema)

and chronic cardiovascular disease (cor pulmonale).

Histology reveals cellular lobules , the relation between

chief cells and sustenticular cells are maintained.



CAROTID BODY TUMOUR

Also known as chemodectoma.

Age: 40-50 yearsClinical presentation:

Slow growing enlarging mass at the angle of the mandible.

Bruit may be audible over the tumour.

Tumour involving hypopharynx may cause hoarseness of voice.

Vocal cord paralysis or Horner¶s syndrome due to

involvement of vagus or sympathetic nerve.

Gross features:Size ranges between 1.8 and 8.5cms.

Solitary, unilateral , dark red to brown in appearance.

Areas of haemorrhage , fibrosis and cystic degeneration .



HISTOPATHOLOGICAL FEATURES OF

CAROTIDBODY TUMOUR

General Features: Overall organoid pattern maintained

Do not have true capsule. Surrounded by connective tissue

with nerves and vessels .

I Cellular characters: Polyhedral to ovoid chief cells with greater pleomorphism

than normal.

Marked pleomorphism in some cases.

Rare mitotic activity.

Cell embracing (enclosing of one tumour cell by another)

Foamy or vacuolar cytoplasmic change (artifactual),

indent small nucleus (resemble lipoblasts).



Cellular characters (continued):

Pseudo-inclusions (like in pheochromocytoma)

Oncocytic change characterized by deeply

eosinophilic,granular cytoplasm.

Sustenticular cells are rare. The cells have ovoid or

kidney shaped nucleus.

Mast cells are numerous.



II Cellular patterns

Alveolar pattern commonly called ³Zellballen´. Organoid pattern

Epitheliomatous pattern in areas of hypercellularity

Plump chief cells are present.

Pseudoacinar pattern

Spindled (sarcomatoid) pattern

Sclerotic pattern -Small nests of cells are present in

sclerosed stroma.

Shrinkage artifact- Retraction of cell nests away from

small vessels. There is loss of Zellballen pattern.

Trabecular and ribbon pattern



III Vascularity

Richly vascular, may give appearance of vascular tumour.

Large ectatic spaces noted. Hyaline change seen around vessels.

Hemosiderin deposits are present in areas of fibrosis

The following are NOT criteria for malignancy: Pleomorphism

Presence of cells inside capillaries.

According to one study, absence of sustentacular cells

correlates with aggressive behaviour .The only absolute criteria is evidence of distant metastasis.



SPECIAL STAINS:

Histochemical stains:Histochemical stains:

Reticulin stainReticulin stain

Accentuate zellballenAccentuate zellballenstructure.structure.

Fine reticulin fibres areFine reticulin fibres are present around present aroundzellballen.zellballen.

No reticulin stain around No reticulin stain aroundindividual cell.individual cell.

Silver stainSilver stain

Cytoplasmic granules areGrimelius positive

(argyrophil)

Immunohistochemical

Stains:

Chief cells:

Chromogranin and neuron

specific enolase- Positive

Sustentacular cells:

S100 protein and sometimes

GFAP : Positive.



JUGULOTYMPANIC PARAGANLIOMA

(glomus jugulare tumour)

Tumour involves the temporal bone.

Most common tumour of the middle ear

More frequent in women (40 - 50years)

The lesion may be related to

-Auricular branch of vagus in the middle ear.

-Tympanic branch of glossopharyngeal in jugular bulb.

Highly vascular tumour with low rate of metastasis.



VAGAL PARAGANGLIOMA

(vagal body tumour)

More common in females.

Irregular mass between the mastoid process and angle of the

jaw in the parapharyngeal spaces. Close relationship with the nerves at the base of the brain.

Patient complains of neurological symptoms, vocal cord

paralysis and hoarseness of voice.

Histologically similar to carotid body tumour.

Dense fibrous band representing vagal perineurium noted.



RETROPERITONEAL PARAGANGLIOMA

Arise from the vestiges of the organs of Zuckerkandl .

May resemble pheochromocytoma or chemodectoma or

both.

Anastomosing trabeculae or twisted cell cords are a

common pattern.

Thin trabeculae of cells are separated by areas of haemorrhage. (Endocrine- like appearance)

Solid or diffuse growth pattern in some cases.

Enlarged or hyperchromatic cells in some cases.

20-40% cases metastasize.

Rarely, melanin pigment present.



OTHER PARAGANGLIOMAS

Mediastinal paraganglioma-

1. Anterior mediastinal - Aortic body tumour.

Usually asymptomatic, detected radiographically.

Aggressive course.

2. Posterior mediastinal ± Paravertebral sulcus

Less common tumour. Nasopharyngeal paraganglioma-

Pulsatile blue masses high in nasopharynx

Laryngeal paraganglioma-

Above level of vocal cord Orbital paraganglioma-

In the ciliary ganglion

Paraganglioma of the cauda equina



Gangliocytic paraganglioma-

Non-chromaffin paraganglioma of the duodenum

Consists of three types of cells:

Epithelioid cells

Ganglion cells

Spindle cells

Bladder paraganglioma-

Trigone (near ureteral orifice).

Invasion of muscularis propria by the tumour is NOT

diagnostic of malignancy.



CARNEY¶S TRIAD Gastric stromal sarcoma

Pulmonary chondroma

Extraadrenal paraganglioma



DIFFERENTIAL DIAGNOSIS

In difficult cases appropriate special stains are used

to establish the diagnosis.

Hemangiopericytoma

Carcinoid

Secondary thyroid carcinoma (specially follicular

carcinoma) Medullary carcinoma of thyroid

Alveolar soft part sarcoma

Other carcinomas

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Paraganglioma INTERNET