CHAPTER Neoplasms of the Adrenal Medulla . MORTON A. BOSNIAK Neoplasms of the adrenal mediila are divided into pheo- chromocytomas (benign ad malignant) and the woore blastoma series of tumor camping neuroblastoma, gan- glloneuroblastoma, and gunglioncurea, The adrenal medulla i formed from cells fom the neural crest, which also gies Hie Bealls that for the Sympathetic chan end pleases! The smmpathete stem also comprises the specialized chromalfir tissue. present ‘mainly inthe adrenal medulla aid in the pare art Zack, erkandls bodies and the exta-adrenaparganglon el system? Because ofthe common deviation ef Us adrenal ‘edillasth the sympathote chan, tumors that oeeurin the adrenal medulla ae alo not uncommonly seen n other retroperitoneal and retrpleural ses. Most investigators agree thatthe primitive cello this serts of mor (ype ae~thogosia) may dilferestiate into a sympathoblas of a pheostromoblast (Fig. 84-1) As they mature and un Gergoifferontition, hese eels gve te, Ia turn, the ‘ympitletic ganglion cell or pheachromosyt (chromaffin cellsNeeplasins develop froin thir chain of ells and the relative maturiy, dilerentation, and malignancy of the lum are determined yi cel of rg. Teorey the cell of ong isa relatively unferentated sympa tobias, neuroblastoma (naligpant wil oreur Hoses AF the cell of oxgin is a well-diferentated sympathetic 7 Decne or OIFERENTION —ncopLasit “issue NEOPLASM rence citerentted ‘Bite ce | ‘ ' uelasama sympaotas! precchomeblst nelson compat) sredoonuntlasona <> _traigae oheteonn) FIGURE 84.1 + The rel { ' Seta dag af ae gg Oovoneors-=—emetberg _peodrenaeye—epteocronsopoma Sent agri Sinponess — Chonate Sy seri eee 2740 84 ganglion cell, a benign tumor will result, the ganglione on Diogreetely Ss coup ae a in Figure 84-1 ‘The most malignant neoplasms aise from the mesg primitive and least differentiated cells and usualy are se Jn early infancy. The tumors composed of the most pip tive cells produce the precursors of catecholamines ng their metabolites. Thus, neuroblastomas are often as ated with a high urinary output of vanillyimandei ag spd oer catecholamine metabalies, The mor ma the tumor is, the less its bologeal activity. The ganglion roblastoma Is more difeentated and less malgnnt neuroblastomas: ganglioneuromas, which are benigt, the most differenGated and mature ofthe series and scl in older children and adults*** Pheochromocytomas Ui dere Fom the pinochronoare zd are uu Malignant pheochromocytomas can occur and often crete a tty of chemi promo of catecelanii sod th table pod, Salar to he ‘neuroblastomas. They may possibly orgiate from ese ferontited cell ia he seres? ‘i ‘An usual hstologieal gradient is exhibited by the nel roblastoma series of neoplasms, and frequently aes, apparently varying. degrees of malignancy are presenti the same tumor. In addition, elements of pleoehroned Frm thentna can sometimes be found in a ganglioneuromas * eoplasis are unigue in that they apparently have vibe ality nge from a clinically and pathologically cpeclgnant tumor to 8 benign neoplasin. This explains the fpeataneous regression and cure associated with this tumor. E eoroblastomas undergo spontaneous rey monly than any other malignant neoplasm by either 3p into benign ganglo Fert e aoocn or nan *Mourbiastomas are discussed in Chapter 86. Because Nemepiemas are found In lder hen and als Ee not covered in other chapters, they are discussed Maar here. Poeodkvomoryiome ere ssceed in Chap ter ‘ GANGLIONEUROMAS Because they are the most differentiated of the neo plasms in the neuroblastoma series, ganglioneuromas are fonsidered benign tumors, occurring much more fre Ee. quently in adults than in children. They may be found in ip jhe posterior mediastinum and in nurherous sites in t GORE 54.2 « Gangloneuroma, agit adzenal. A Neoplosms of the Ackenol Medula +++ 2749 isopertoneum, pis, atest at and mee tery tna seer oF 5 cate of gangioneurene the tomor was lente nthe abdomen in poe than hao the cages Ee) tw the medastuin i 09% of eases, and in the pala or neck i S%* Of thse gnghonsuronaseccrring Eithe cemopertoneur, about cnet eur n the adres false Theor, approuatey 25% of unglonetromes Are situated in the adeonel glands “thee beng neopasnstare pial sow growing and renin clnieally glost f located ina poston that doesnot {Sane presuro poms, Net neq they are found By alunos Guten imaging study, When they are symp- Tae the fangs they oduce depend on thelr se tod lbeaton, so tht on cczson, the cinta presentation 1 Ghat of aadoinal pain oy mas, Adrenal gononeuromas Gaually ensure spproxiataly 8 to 15 em in Gameter when fiseotered resi unary levels of catecholamines are some umes sean pao wh gangloneucomas ni may have Sdvenerge syoptoms sucks clertben, hypertension, and Secale Te becase. gafmeuromas, cooslonally centas a compente tuner wih. pheochromocytom rab the gunghen cells of he tumor can produce &2750 ++ The Actenal Gianes FIGURE 81-3» ae inherit se se rma Sage diontrone ame ef ee ube Dt ‘atoactive intestinal polypeptide. that is associated with diarrhea." Ganglionenromas are also associated wit lignant peripheral nerve sheath tumor and also on occasion contain elements of ganglioneuroblastoma and neuro Dlastoma.” " ‘Therefore, althoyeh most ginglioneuromas fare asymptomatic. incidentally found masses, their associa tion with these hormonally producing ane malignant lesions helps to explain the occasional unusual symptomatic pre sentation of these tumors” ‘Adrenal ganglioneuromas appear as space-occupying FIGURE S14 card woman wt is well crennserbe ol et in ht of te eB, Transverse TBaveghted MU eum (AWHVIS3) does tat te mse hele gil nal reveled se araph: Ultras exurinaton reveal 9 relate lucent he i iageneous tumor (Fig. 84-2). On computed smography (CT), ganglioneuromas usually upped at woh snarginated masses, often lobular in appearance. They rg homogenous and relatively low attenuating (less than roe de) on the non-enhnced sean, similar to what has bea described in extracranal nerve sheath tumors" <= Gey Fig. 64-2). After contrast enhanceiment, most lesions a main homogeneous with relatively sight enhancemen sully Igs than muscle wssue (ee Fig. 843: Fig. 86-35 However some lesions enhance heterogeneously. ed exe, sionally areas of higher attenuation thin the tumor. pies" In appre 0% of cen puncte ca Feations are present Anglographicily, they are hypo cular" Maghetie resonance hnaging (MRI) fading they 4 homogeneous. low-ignal (less than Hiver)lsionon Ty ‘weighted sequences. On T2aveighted scans, the tumor is charactristeally heterogeneous with ‘high signal that greater than that of lver’® (ig. 8-4), The deg of heterogencity on T2-weighted MAY is much greats tht that seen on the enhanced CT images. On gadeliniua, enhanced MRI scans, one sees moderate enhancement tf the tumor. On dynamic scans, it has been reported tat Jack of enhancement occurs eat after injection, but wi a grulnal increase in enhancement on dltyed scans These tumors are relatively soft snd therefore are more spt io change shape to accommodate tothe space in which they are growing ond ae les key to ophee ter organs. This explains why most ganglioneuromas of the Fight adrenal sare om, conforming te the space benweeh the Iver and the spine: This shape is nicely seen in Figures 84-2, 43, and 8-4 tl dseomfor, A. Tranwene Tha ‘63 Ten The mass sight npper ule a Fram Ran R. Dai. cal il gg Timing Bing 13 as Rajnsummary, the CT and MRI features most characters ge uf ganglioneuromas are (1) a well-defined oval mass fe a Storms to the space it occupies, (2) low attenuation tht femogenelty on non-contrast CT scans with only mod talenhancement, (3) dserete punctate calcifications, F(a) nonhomogeneous but high intensity on 12. piped MRI scans and delayed enhancement pattern on pains REFERENCES | Baral DS, Rubinstein Lf: Pathology of Tamore of the Nervous Sytem, th ed. Balimor, Wiliams & Wilins 1989 4. Nena KL The endoelalng fanction of relstedsntacide: Cate ‘isin, setplcholne, seein and hia, fa Wilas REE {alt Teubsak of Endocrinology, Gth ed, Phadlpis, WB Saunders, fos, pp S158 4 Kare HT Tarors of the adrenal, In Kassner HT (ed: Ath of ‘Toor Pathology, Bod sees Bethanda MD, Armed Fores inte {1 Tumor Pathology. 2080, pp 253-260. Lsberg Ly Young JB: Catechlamines ard the adzenl modu. 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