(NON-CHROMAFFIN) PARAGANGLIOMA
EXTRA-ADRENAL PARAGANGLIOMA (DERIVED FROM NEURAL CREST (APUD) CELLS)—–NEURAL CREST CELLS–MELANOCYTES, C-CELLS OF THE THYROID, SYMP. CHAIN, ADRENAL MEDULLA, CRANIAL NERVE PARASYMP 3,7,9,10
CHIEF CELLS IN CLUSTERS OF ZELLBALLEN, SUSTENTACULAR CELLS, OXYPHILIC CELLS—S-100+
DESCRIBED BY ROSENWASSER 1945
FEMALE 3:1
L>R
WHITE>BLACK
5TH-6TH DECADE
LIKE ADRENAL TUMORS (PHEOCHROMOCYTOMA)—RULE OF 10’S
REALLY A RULE OF 1-10% DEPENDING ON SOURCE
10% MALIGNANT—-MET TO LUNG, BONE &LIVER
10% MULTICENTRIC
10% CATACHOLAMINE SECRETING—MAY ALSO SECRETE CHOLECYSTIKIN–POST OP ILEUS
10% FAMILIAL–CAN BE PART OF MEN SYNDROME—ASSOC WITH ANOTHER MALIGNANCY
MEN IIA (SIPPLES SYNDROME)–AUTO D–RARE—BETTER Px
BILAT PHEOS–BEWARE OF CATECHOLAMINE!
BILAT MEDULLARY CA
PARATHYROID HYPERPLASIA –HYPERPARATHYROIDISM
MEN IIB–RAREST–WORSE Px–MORE COMMON IN CHILDREN/ADOLESCENCE-(YOUNGER AGE)– 3RD DECADE
AUTO D OR SPORADIC
BILAT PHEOCHROMOCYTOMAS
BILAT MEDULLARY CA
ASSOC MUCOSAL NEUROMAS OF THE ANT. TONGUE, CONJUNCTIVA, TARSUS, LIPS, BUCCAL MUCOSA
GANGLIONEUROMAS OF THE BOWEL—MEGACOLON
MARFANOID HABITUS–LONG EXTREMITIES, HIGH ARCHES, KYPHOSCOLIOSIS
RARELY HAVE PARATHYROID HYPERPLASIA
Dx:SERUM CATACHOLAMINES, 24 HOUR URINE [VMA, MHPG, METANEPHRINS], CAN DIFF BETWEEN GLOMUS CELL TUMORS AND PHEO BASED ON FACT THAT GLOMUS LACK THE ENZYME METHYL TRANSFERASE AND CANNOT TRANSFORM NOREPI INTO EPI—-THESE ARE COST EFFECTIVE AND RESONABLE TESTS
FLUORESCENCE OF FORMALDEHYDE-CONJUGATED CATACHOLAMINES—”HOT GUANO SCAN”
CT/MRI WITH CONTRAST—-DSA (DIGITAL SUBTRACTION ANGIOGRAPHY)
GET A TSH TO R/O HYPERTHYROIDISM
ANGIOGRAPHY-”EGG SHELL LIKE MASS”—-MAY EMBOLIZE AT SAME SITTING IF LARGER THAN 3 CM
CAROTID BODY TUMOR = CHEMODECTOMA
MOST COMMON PARAGANGLIOMA OF THE H &N
FROM CN 9 (NERVE OF HERRING)—-CHEMO AND BARORECEPTORS AT THE BIFURCATION
BLOOD SUPLY MAY BE FROM THE VERTEBRAL, TCT, OR CAROTID
SLOWLY ENLARGING, FIRM, RUBBERY
LATERAL MOBILITY WITH NO VERTICLE PLAY
PULSATILE
+/- BRUIT OR THRILL(PALPABLE BRUIT)
COMPRESSIBLE BUT RAPIDLY REFILL—-BEWARE OF RESULTANT HYPOTN (NERVE OF HERRING)
WARM
MEDIALLY DISPLACE THE PHARYNGEAL WALL
ANGIO—–”LYRE” SIGN = EGGSHELL LIKE MASS CAUSING SPLAYING OF THE CAROTID BIFURCATION AND “MAYER’S” LIGAMENT WHICH ENVELOPES THE BIFURCATION
Rx: SURGICAL RESECTION—–BE PREPARED TO GRAFT—10% RECURRENCE—–PRIMARY NERVE TO INJURE = 12
JUGULOTYMPANIC PARAGANGLIOMA———-MOSTLY GLOMUS JUGULARE
2ND MOST COMMON H &N PARAGANGLIOMA
MOST COMMON TUMOR OF THE MIDDLE EAR
3 GLOMUS STRUCTURES IN THE T-BONE
FEMALE 5:1
MEAN AGE 52
BLOOD SUPPLY = ASC. PHARYNGEAL ARTERY
L > R (OTHER STUDIES SAY R =75%)
INSIDIOUS ONSET OF SYMPTOMS-PAIN, CHL -PULSITILE TINNITUS–(DIFF Dx IS BENIGN INTRACRANIAL HTN, VENOUS HUM (TURBULENCE IN THE SIGMOID), VASCULAR ABNL–HIGH RIDING JUGULAR BULB>INTRAPETROUS CAROTID ABNL)
+/- BRUIT = OBJECTIVE TINNITUS
28% + BROWN’S SIGN = REDDISH BROWN (VIOLACEOUS) HUGH WHICH BLANCHES WITH POSITIVE PRESSURE ON PNEUMATIC OTOSCOPY
RADIOGRAPHIC EROSION OF THE COCHLEAR KEEL–ENLARGEMENT OF THE INF TYMPANIC CANICULUS, ENHANCING MASS IN THE HYPOTYMPANUM, ABSENCE OF BONEY COVERING IN THE INT CAROTID AND ABSENCE OF ITS VERTICLE SEGMENT
GLOMUS JUGULARE—-OFF JUGULAR DOME—JUGULAR GANGLION OF CN 9
PRIMARY SITE FOR MIDDLE EAR GLOMUS TUMORS—–BY FAR!!!
GLOMUS TYMPANICUM—OFF TYMPANIC PLEXUS ON COCHLEAR PROMONTORY
THE SECOND MOST COMMON SITE IN THE MIDDLE EAR
GLOMUS VAGALE (INTRAVAGAL PARAGANLIOMA)
3RD MOST COMMON H & N SITE
PRIMARILY AND THE GANLION NODOSUM OF CN X
MAY DUMBELL INTO THE JUGULAR FORAMEN—CAUSE VERNET’S SYNDROME (CN 9,10,11)
SLIGHTLY HIGHER INCIDECE OF DISTANT METS (19%)
OFTEN HAVE TO RESECT CN X—NEED TVC MEDIALIZATION
Rx: EARLY SURGERY IS BEST—-BE READY FOR VASCULAR SUPPORT (CAROTID NERVE GRAFT) ON CAROTID BODY TUMORS——8% MORTALITY AND A 10% RECURRENCE—–THINK ABOUT PRE-OP EMBOLIZATION (48 HOURS)
RT MAY PALLIATE BUT ARE PRIMARILY RADIORESISTANT—-SOME REPORTS IN THE LITERATURE OF CONTROL RATES 70-90% WITH RT (3500-5000 CG)—-RT PRIMARILY AFFECTS THE VASCULAR AND STROMAL ELEMENTS—NOT THE TUMOR CELLS
Approximately 20-30% of pheochromocytomas/paragangliomas are associated with a familial hereditary condition. Multiple genes have been implicated – including von Hippel-Lindau (VHL) syndrome, neurofibromatosis 1 (NF1), and multiple endocrine neoplasia, type II (MEN2). In addition, familial pheochromocytomas/paragangliomas have been linked to mutations in the succinate dehydrogenase (SDH) genes, most commonly SDHB, SDHD, and SDHC.