OREGON PATHOLOGISTS ASSOCIATION Pediatric Slide Conference

LOUIS DEHNER, M.D . June 7, 1980

CASE 1: UNIVERSITY OF OREGON HEALTH SCIENCES CENTER (Dr . Karen Ireland)

('\ '\ ... jq~79-S-7504): This is a three year old white male .\' \ 1v 1 with a one month history of abdominal 1 pain, increasing abdominal girth, hyperperistalsis and

decreased appetite . He presented with a large abdominal retroperitoneal mass involving the right colon .

CASE 2: UNIVERSITY OF OREGON HEALTH SCIENCES CENTER (Dr.Karen Ireland)

(79-S-7377):

duration which was also a two the left side. resected.

This is a six year old male with chest pain of approximately one month's radiated down left arm to back. There month history of Horner's syndrome on

A posterior mediastinal tu~or was

CASE 3: ~NIVERSITY OF OREGON HEALTH SCIENCES CENTER: AG 1 (Dr . Stephen Kessler)

~{ J"'-(\0 (77- S-1866): This is a sixteen month old white

\~ female with a six month history of a mass in the left lower anterior right thigh .

CASE 4: UNIVERSITY OF OREGON HEALTH SCIENCES CENTER: _ {,, (Dr . Stephen Kessler)

.\

'

V C(i"\ L\~~' (77-S-&,~J.) This is a. fourteen ye~r old ~emale with ~~~ an expans1le destruct1ve les1on of

~-·• the left maxillar y sinus, hard palate and nasal cavity . ~

~ ,r CASE 5: UNIVERSITY OF OREGON HEALTH SCIENCES CENTER (Dr. Donald Houghton

(S-19-79) This is a rapidly growing subuctaneous right cervical mass in a fourteen year old boy. '

OREGON PATHOLOGISTS ASSOCIATION Pediatric Slide Conference

LOUIS DEHNER, M.D. June 7, 1980

I CASE 6: UNIVERSITY OF OREGON HEALTH SCIENCES CENTER ~\ i (Dr. Donald Houghton)

(' ..1 · \ -"l/:U\ ~~U _/ ·s 2116-76) This is a right r enal mass in a neonate .

CASE 7 : UNIVERSITY OF OREGON HEALTH SCIENCES CENTER ~1) (Dr. Donald Houghton)

~~--~ s 6275-76 This is an eighteen year old female \ '~ with bilatera l renal tumors.

CASE 8 : /

~()f)l? PORTLAND GOOD SAMARITAN HOSPITAL (Dr . Eric Jacobsen)

(A-79-103} This is a neonate wi th multiple congenital anomalies predomina ntly

involv ing the lungs and genitourinary system .

CASE 9: VANCOUVER ST. JOSEPH COMMUNITY HOSPITAl

~~ :::~2::::~e Ham:~::n:~ a four year old Greek male who 0~\J'~ prese nted with a fungating lesion of ~ the left eye.

CASE 10 : EUGENE SACRED HEART GENERAL HOSPITAL \v (Dr . David Rutz)

r-A~ (431463) This is a ten year old Caucasian male with a ~··~ · large mediastinal mass extending into the ~ left pulmonary hilar area compressi ng the left main ' stem bronchus .

CASE 11 : PORTLAND KAISER FOUNDATION PERMAMENTE HOSPITALS (Dr.Katherlne Holahan)

.\ '

~~~\~~(S 39952-77) This is a seventeen year old female who 1\v presented with a rapidly growing mass in

the region of the upper right a rm near the axilla . An excisional biopsy of a soft tissue tumor measuring 4cms. in max imum dimens ions was obta i ned .

) CASE 12 :

- '~~ ~)

OREGON PATHOLOGISTS ASSOCIATION Pediatric Slide Conference

LOUIS DEHNER, M. D. June 7, 1980

PORTLAND KAISER FOUNDATION PERMAMENTE HOSPITALS (Dr. Kather ine Holahan)

(S 3302-79, S 5950 -79, S 37203-79, S 41221-79 ( s 6973-78)

Pigmented skin lesion of a child .

(3)

FOC/jv 7687B

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OREG011 PATHOLO!;ISTS ASSOC I AT !Oil

DIAGNOS IS AND REFEREtiCE

.36S

~. t..o U CS 'ldt,/+uE)'2.. -.lui.Jt ·1 q $' 0 J

Case 1. r~a l ignant lymphoma , small noncleaved cell (so-called Burkitt 's l ymphoma) .

This neoolasm reoresents the co~onest orimarv malionant tumor of the intestinal . . . .. .

tract in childhood. The only possible source of confusion is 1·iith nodular lymphoid

hyperr 1-asi a ·of the term i na 1 --Heum~cecum but -t he d-i 'f-ft~se i nf.Htl"a t ·i·ng -nat<~re af the ...

lymphoma and extension .to th.e sero'sa are features of mal.ignancy. Reac:ti ve germ·inal

centers are totally absent. Focally, a "starry-sky" oattern is noted but it is

not specific for Burki tt•s - l ymphoma. It is ·also present l'lith T-cell (lymphoblastic)

lymphomas in children and al so metastatfc tumors such as l ymphoeoithel iofl!a and.,

rhabdomyosarcoma.

The now International Cl assifi cation for non-Hodgkin' s lymphoma (1980) designates

this tumor a "'small noncleaved cell lymphoma" but the cells are larger than the

small lYfl1phocyte. It is well established that this tumor is not "undifferenti ated"

bu.t is a 8-cell neopl a.sm i n which t he cells have monoclonal surface immtJnoglobulin

of the Igt1 c·lass . Sel ect ive i nvolvement of germinal center s by Burkitt 's lymphoma

11as demonstrated by 1·1ann et. al . and thu~ thi s tumor i s the cliildhood eq'u'i valent

o( follicula·r, small cleaved ce11- lymphofTia ~if adults . The rapid proliferation of· ·

the Burki tt cells fails to permit this ,attern of the disease to become established.

A chromosomal aberration in t he' form of chro~osome ·14q~ has· been reported in the African and non-endemic cases. It. is t hought t hat t he EB vi rus is someho;.¥ responsibl e_

for this anomaly.

One area of morohologi c confusion is the existence of "non-Burkitt's" small

non-cleaved. cell lymohcima. The tumor c~lls ~re characterized. as more pieomorphic . . than t he uni f orm appea-rance of typi ca.l Bur kitt's lymplloma. Hhen these non-1!urkitt • s

cases are examined in terms of cl inical presentation, marker characteristi cs and

prog!'losis, the~e ·~·eems ·littl e.- just.ification t o separate theni. PersonaliY.. t believe

that some of t he ·putative pleomorphic changes are the resu)t of poor fi;.cation and

t hick secti oning. . .

References

1.. Golding RL, et al .: The radiology of gastrointesttnal Burkitt ' s lymohoma in

children. Clin Radial 28:465, 1977.

2. Kaschula RO, et' al .: Immunohistochemical characterization of Burkitt' s l ymphotr.<! .

S Afr ~led J 53:655 , 1978.

i' ·~

·-·--·- .... . - ·1 . .. ._..:..__·---:-, ___ :__ _____ , _____ ~--r-------...,...----;..,.----·-......;_,· ·

TYPE

·SHALL L Yt·lPHOCYT I C

. FOLLICULAR 1 PRE DOHINANTLY SWILL CLEAVED CELL

' GRi\DE

FO!.LICULAA 1 I.UXED (SMALL CLEAVED AND LARGE CELL) . .

SUBTYPES OR VARIETIES

c;w·cLL Pl a~ma.cy toi d

' Wi t h d i1f~se nrcas With scleros'is '

With diffuse areas l~i th ·scJeros is

----~-----~--~------------~~---------------------~ } . ' : f'Ol:Ll CULAR 1 PREPOMI NANTL Y LARGE CELL ; \~i t h di ffuse area.s ' '

I 1\TER- \~i th sc.leros i s . 0 I FFUSE 1 Sl·iALL CLEAVED CELL ~/i t h sclerosis

;.:£!H ATE DI !:FUSE 1 14! XED ( St•1ALL AND LARGE CEll) \.Ji t h scleros i s

GRADE I.Ji t h epithel ioid cell component : ' •

Gl eavcc.J ce 11 Non·- clea v~d ce l l·

'DIFFUSE I Li~RGE CEL,L

With sclerosi s .

LARGE CELL 1: ll'il•lUNOBLASTIC . Pl asmacytoid ' Clear cell

HIGH . Po f yino r p hou s Hi th; epithel ioi d cell component

. ' . ' '

' '

L YNPHDBLAST.IC Con yo 1 ut~d ce 11 GR.t\OE Non-convol uted cell .

' SI·1ALL NON- CLEAVED CELL . Burki tt ' s With fo l l icul ar areas

I '

~4~~~----~--·------~------------~------------II----~--~----------~----J

I

. . ~~~ SCEL Li\NIOUS .

I

Compos ite . t·lycos is Fun go1 des

; Hls t iocyt ic · Ex·~; a~i~~ ~lu i 1 ~pi P1 a s n;acy t ollla :_

.

.·

2

l·la 1 i gnant 1 ymphoma - a conce!)tua 1 understanding

Pathol 94:105, 19/9. - .

of morphologic 3. Hann RB, et al .:

diversity. Am J

fo\ann RB, et· al .. : . . 4 . . Non~endeini c \3urki tt ' s lymphoma . · P. B:..ce 11 tumor re 1 a ted to

germ'i·na '\ centers. N ·Eng'\ J i~ed 29S :o2.5, '\976.

5. Phi'\i~ P, et al.: Harker _chromoso:r~ l4q+ in non-endemic Burkitt 's iymphoma.

Cancer 39:1495, 1977.

6. Templeton AC: Changjng pattern of residual tumor in Burkitt's lymphoma.

Arch PathiJl Lab ~led··l 00 : 5-03·,- ·1976: --~ ..

7. Zej9]er ·J_!.: .Treatment_ res.ults of 54 American patienti \'lith Burkitt ' s lymphoma

are s-imilar to the African ex!)erience. N Engl J !·led 297:75, 1977.

8. Zeigl er JL, et al . : Ebstein-Barr vfrus and human·w.alignancy. Ann 'Iritem ~led

56: 32 3 ' 1977.

Case 2. Posterior medi astinum- malignant sch•11annoma.

It is strongly suspected ~hat this child has vo~ Recklinghausen 's disease and

that one of the plexiform neurofibromas has undergone malignant transformation.

Thi.s child is on the young si·de for this compl ication but· there .are purported

exam!)l es of congeni ta 1 rna 1 i gnant schwannomas. At 1 QVI power; "the· ci_rcuf!l~_c,ri bed

nature of the multiple !)lexiform structures ~s optimal ly· appreciil:ted. A fibro-

hya 1 ine stroma · separates these structures.' There 1-1as also evidence of focal necrosis.

Some of the plexiform structures 1-1e·re quite cellular and at hiqher po:;er, mitotic

activity and pleomo~phism were -noted . . The latter finding is not a h1ays. indicative

of malignancy and in f act, it is not ·that uncommon in solitary schwannomas ~lith

other featuces of degerierafi on. Nitotfc -activity fn. contrast is very ·significant

in terms of mal ignancx. It. is inter.esJin·g to note that not all malignant schwannomas.._

will show uniform mitotic activity but it may be very focal and thus in a large tumor,

it i s very important to have: multfple sections.

The histo!)a thologic diagnosis of .malignant schwannoma is not ah~ays a simple •

one s~nce it is basically a spindle cell . sarcoma to be d~fferentiated from fibro-

sarcoma, leiomyosarcoma,. monophasic synovial sarcoma and hemangiopericytoma. It . .

is an especially difficult diagnosis in the !)atient with the solitary malignant

sch11annoma (70-75% of all cases in some sertes). In the la tter case , the i"elation­

ship to a major nerve is virtually imperat i ve. Hhen the soft tissue sarcoma occurs

in the individual ~lith ~on Recklinghausen's disease, vi~tually any. microscopic

pattern becomes acceptable as a malignant schwannoma. Interestingly, there is a

striking difference in the 5-year survival .beh1een the sol itary categor>' (65-7Sr. )

3

and t he vo_n Rechnghausen' s group (25-3mn . Ac;_cor di ng t o Ghosh et al. , a ma 1 i gnant

.schwarinoma ari si'ng i n a p 1 exi form neu rof ibr oma ha s a wors e prognos i s . -

An int eresting bio1og·ic phenomenon in mal i gnant schviannomas,esoecially in- .

patient s 1·1itp '{gn R'eck jnghau~en' s disease,. i s the presence of h~terol ogous structures

such as mucinous g1 and_s ,_ rha bjJomyob 1 asts (' 'Tri-t on t umor" L car ti 1 age and even bone ..

There is apparently no prognost·ic si gni f icance to t hese addi ti onal ti ssue types

iii the t umor. ·

Finan y , among soft tissue sar coma,s i n c_hi.'l dhood ; ·the ·ma.lignant sch\·t<mnoma

does not constitute any more than 5% of the total number. . '

Refe.r ences .\

1. Chen KTK, et .al.: Malignant schwannoma. A light microscopic and ultrastruct ural

study. Cancer 45:1585, 1980.

2. Ghosh BC, et al. : Mal ·ignant schwannoma. A clinicooathologic s t udy. Cancer

31 :184, 1973 .

. 3. G,1.1 ccion JG ang Enzing~r Fl1: Ma1i~mant s_ch\~annoma as~oci ated . with y_on R.eck­

lin~hausen's neurofibroma~osis. Virchows Arch (Pat hol ~nat) 383: 43, 1979.

4. \-lander JV aQd Oil,s: Gupta TK: Neurof ibromatosis. Curr Pro~l Surg volume 14 ,.

•1977 . - . ..

5. Hood·ruff J t1: i>eripheral nerve tumors sho1~ing glandular diff erentiati-on

(gl anclul ar schwannomas}.· Cancer 37 :2399, 1976.

6. Hoodr~ff JN, e_:t a l. : Peripheral nerve tumors

di f ferentiation (.mal igLJant "Triton" tumors).

with rhabdomvosarcomatous . - " . Cancer 32:426, 1973.

Case 3. Soft t-issue · - Fibromatosis l~ith -fea~ures of fibrous -hamartoma o-F -infancy.

A poorly define_d mass in this infant is compesed of bands, septa and small

islands of fibrous tissue I·Jhich shows some variation in the cellular. Some o{ the

bancfs a.re quite cellu]ar \•lhereas others contain more coll agen_ as· evidenced by - -

the -background eosinophilia. -Scattered sma·l l islands are very r-eminiscent of the

so-called neuroid bodies described in t !1e fibrous hamartoma of in_fancy. · Th~

adipose tiss.ue as an. indigi nous par t .of the neoplastic process is very di fficult'

-.

to 'access because this tumor is located in the subcutaneous ti ssues.· It is f airly

mature fat ;therefore ,I do riot believe that 1·1e shoul d seriously cons-ider a lipoblast -

. oma although in -a yo.unger child, the di.fferentiation of a lipoblastoma from a

- · fi b·rous hamartoma is not an easy t ask in every case. There is an important

prognost ic di f ference sil)ce .the fibromatosis is more likely to r t1cur than the

1 if)oblastoma,

4

Althou9h some refinements have occurred i n .the classi fication of fibromatosis

and t he recogni tion of some specific subtypes , there remai ns a great dea1 t hat

remai ns unknown about this- enigmatic group of neoplasms. One of' the paradoxes

that exist is the discrepancy bet1·1een the microscopic aooearance and the clinica l

behavior. This is best illus~rated by the congenital fibrosarcoma - like fib ro­

matosis \•lhidi i n many respects looks l i ke a fibrosarcoma in an adu l t yet' thi s

tumor- rare 1 y -metastas:i zes -desp'ite the abundant mi tet<i c activity. ·- · · ·

Ther E:l are particvl ar subtyres of fiBromatosis i n ch.i i ~hpoiJ 1·1ith djs·tinctive ...

microscopi c features 1·1hich permit separation from the "not othen·lise specifi ed"

fibromatos is. ·The fibrous hamartoma of; infancy' is one of these. ' Uri like · the'

other types of fibromatosi s , it contains adi pose tissue as wel l as t he peculiar

l ittl e profil es of f ibrous ti ssue resembl ing neural bundles. I n ou r experie nce,

t his t ype of fi bromat osis i s one of t he l east common. The other distinct ive forms

of the disease include di gital fibrous t umor (also desc ribed by Re_ve as 1·1as the

fibrous hamartoma}, juvenile aponeurotic fibroma and fibr?matosis hyal inica

mult i plex juve~ilis .

The kev ooint i n 'the management of these l esions i s 11ide l ocal exci s i on. 1·1hich ~ . ' .

i n order to- be successfu l mus t incl ude margins 1·1ell beyond t hose judged by t he . . . · surgeon as free of involvement. There i s ·t _vp-ically·ins idious spread beyond the

main mass into the adjacent skeletal muscle and f~t.

Refer ences

1. Allen- PH: The fi bromat osi s : a cl inico!)athologii:. class'ifi cation based 'on 140

·cases. Part 1. Am J Surg Pathol l :255; 1 gn . -

2. Allen PH: The fibromatosis: ·a clinicopathologic classification based on 140 •• cases . Part 2. ' P..m· J Sur9 Pathol 1:305, 1977 .

3. DehnE:r. LP ancl Askin FB: Tumors of fi brous tissue origi n in ch'ildhood.

Cancer 38:888, 1976. 4. Rosenberg HS et al. : The fibromatoses Of infancy ·and chil dhood. Perspect

Pediatr Pathol 4:269, 1978. 5. \-lee A, et al.: Infantile fibrosarcoma . Report of cases. Arch Pathol L?b

Med 103: 236, 1979.

Case 4 . · Maxillary si n-us - giant eel~ granuloma.

I first had the opportunity to revie·.~ this Clm! in 1977 as a consultation

and needles s to say, it was a very per~ l exing prob 1 em. The x-ray r evea 1 s a ~arge

5

soft tissue oens it% fi lYing t lie antnim and P.roduci ng cons ·i derab 1 e destruction .

It was not unreasonabl e in sucli a circumstance to strongly consider some type of rna l i gnant les ion of mesenr:h.yma l derivati on . _ i1i c.ros.copi CiJl ly, the J;um9 r h~s. i;l ..

spindle cell stroma which in most areas. is quite. compact. Ni totic activHy .i s " -- - . - . ' .

not abundant nor is t here any pleomorphism. A storiform oat tern ·is al so suggested

in" some areas and therefore, · a fibrous h·i s tiocytoma with ' lm'l grade malignant potential'

~/a s one. of .ma.ny tho.ughts. Other fi.elds of this tumor conta ined bland· appe.;·r-ing

multi nucl e.ated cel l s, some of 1·1hi ch were aggregated about spaces or lacunae in

the fissues . These l"acuiiae 1·1er'e rtiled- 1·1ith eryttirocyfes . or serum: At .th.is~ point~·

my di fferenti al diagnosis shifted from t he fibrous histiocytoma, lo•.., gr.ade f i l>ro­

'sarcoma or aggressive fi bromatosis to that lesion known as the giant cell granuloma

.or fo rmerly the giant cell (reparative) granuloma. Nost giant cell granulomas are

small er {less than 2 em. ) and located in t he post er ior mancl ible,, if central, or

as a ' poJyRoid mass on the gingiva (gi ant cell e!)ulis or p.erioheral giant cell

. granuloma). As this case demonstrates, thi s -lesion may become l(juite large and

locally destru-ctive ·yet does not demonstrate mal i.gn.ant behavior. Thi s.. same type

of -process may involve al ,- four quadrant-s of the ja1~ and is transmitted ·as an

autosomal dominant t rait (Cherubfsm). Hhen a· qia.iit.c·en granuloma has at tci'ined··

tlie "dimerisiori; of t he pres~nt case, total ecxt irpation is very' difficult and thus

recurrences a~e not ·surprising. Surger:y is the treatm_ent of choice aTlld some ti_ave

stated t hat .irradiation is contraindi cated.

The different-iitl di.a,gnosis i ncludes tho_l;e oj:her_lesions in the ja:ws and - ... . .. . - ..... paranasal sinuses ~lith giants including the very rare giant cell tumor (osteocl~stoma ) . .- . . "brown tumor" of hyperpara'thyroidism and aneur.ysmal hone cyst. It is not oossibTe

in my experience te dHf.erentiate on the basis of hi stology th~ "bro•.m tumor" f rom

giaht cell granuloma·. . . Histogeneticall'y, the _gi ant cell _gr~hulol)!a is t;()mP_9 ?,ed 9f. ~istio_c;ytes. ~(ith, .

phagocytic activity. These · cells seem to emerge from the fibrous str~ma .. . It

may \•Jell be that the gianl cell granuloma is a variant of the fi brous histiocytoma

or alternati vely a lesion of .myof ibrobl.asts .

References

1. ·Adkins KF, et _al. : Ultras t ructure of giant-cell les ions. Oral Surg 33:775,

1972. 2. Bat s·akis JCi: Tumors of t he head and neck. Clinical and patnol ogical con­

siderati'ons. 2nd Edition . Baltimore. \~illiams and \~ilki ns, 1979 , p. 396-397. . .

•

6

3. Dehner LP: Tumors of the mandible and maxi lla in children. I. Clini co­

pathologi c study of 46 h·istologically ben ign l e sions . Cancer 31:364, 1973 ..

4. Schlor·.f RA and .Koop SH: 11ax-i ll ar.v g·iant cel l re!Jarativ~ granul or.1a. LarynC)o­scooe 87: 10, 1977.

5. Smith GA and 1·/ard PH : Giant-cell lesions of the facial skeleton . Arch

Otolarynqol 104:186; 1978.

6. Haldron CA and Shafer. HCi : The cer-~-tra l giant cell · r epara-t ·ive ·<;Jranul oma· of -the:

jaw. ~ Am J Clin Patliol 45:437, 1966. - -- - . . Case 5. Lymph node and/or .• soft tissue - ]YJilphoepi.th.eliorna (poorly differentiated, _

nonkeratil) izi.ng squamous ce_ll carcinoma), metastatjc .

The biopsy unequivocally shows a mal ignant tumor composed of cohes5ve sheets

and a 1 so smaller nests which are del ineated from the surroundind infl ammatory

cells. · !•lhat are the various reasonable possi bilities based upon age and location?

The ones that come to mind are malignant lymphoma, large cell type (histiocytic),

lymphoepithelioma, malignant hi stioc:ytosjs, Hodgkin's disease, .r:habdomyo~arcpma

or a met astatic. germ c·ell t umor, in pa-rticul ar embryonal carcin~ma .. Some of these

more eas i ly dismissed t hi!n others . Rhabdomyosarcoma is not comPa tible 1•1ith

11hat we are seeing and l ikewi se Hodgkin.'-s disease seems unlikel y . If you noted,

there 1-1ere focally mariy eosinophils present at the periphery of the neoplastic

nests . Embryonal ~arcinoma is no~ very high on the l i st from the clinical stand­

point but mi croscopical l y, t he sol id arr~ngement qf ~umor ce ll s and the pl eomor phism

. and .atypja. a re .compati ble findings. The tumor . i~ - t ao cohesive fo r malignant

histiocytosis but I have recently encountered a case in a child where the diff~rent­iation \'las less than ~clear. I believe that this case is a nice examole of 'the . . .. . . . so-called lymphoepithel iOf!la with the S<;_hmincke pattern _of th_e intermi xt ure of

t umor cells wi th il)fl ammatory cell s. J he Regaud pattern i s characteri' zed by dis~.

crete nests s-eparated by fi b·rous . stroma producing a distinct epithelia: 1 appearanGe.

It is typical for this tumor to present 1·1ith cervical _lymph node ll]etastasis from •

a sma 11 tumor in the nasopharynx. J.n one series, 40 of 42 patients presented liith ·

a neck metastasis. The princ·ipal histol ogic !JrOblem i s· di .fferentiat!n_g this

tumor f rom l arge cell (histi ocytic) lymphoma. Light microscopy may oc casi onally

fail but in most cases, the correct diagnosis can ·be made.

'As 1·1ith Burkitt's lymphoma, there has been a great. deal of interest in the

role of the. Epstein-Barr virus in the etiology of thi~ disease . Some epidemiologic

features of thi s tumor . al s o suggest· an infectious or at least environment agent(s)

~ .

7

are involved .

Re·feren ces 1. Deutsch t-t, et al.: Cancer of the nasopharynx in chil dren . Cancer Ill:

11 2$ , 1978. 2. Fu Y-S and Perzi n KH : Nonepithelial tumors of the nasal cavity, paranasal

sinuses and .nasopharynx. A cl in.icop.athologic. study. X. 14al i gnant l ym~hor..a . .. Cancer 43 :611, 1979. -

• • • 4 •

3. Giffler RF _et al . : and young . adults .

Lymphoepithelioma in cervical lymph nodes of chi l dren Am J Surg· Pathol 1:293, 1977.

4. Greene ~lH ·et ·al .: Nasophary11g'eal cancer arr.ong young 'people in the Un"ite4 -States: racial vari atiqn~ by cel l type. J Natl Cancer Inst . 58 :1267, lg7].

5. Henderson BEet al.: Epstein-Barr virus and nasopharyngeal carcinoma: is there an etiologic relationship? J llatl Cancer Inst 59:1 393, 1977 .

6. Shanmu~aratnam Ketal.: Hi stopathology of nasopharyngeal carcinoma. -

Correlations ~lith epi_demiology, survival rates and oth~r b_iologica~ character-i stics . Cancer 44:1029, 1977.

Case 6. Kidney - mesobl a's tic nephroma·.

This spindl e cell tumor of the kidney is the commonest intrarenal neoplasm in early infancy, less than 2 months olrl. It i s one of the neopla sms that Bolande . . has designated under the rubric, infantile r ena l neoplasia includ ing nephroblast­omato.si s and bil_ater al Hilms' tumor.· The early report s before Bol.a.nde et aL 's publ i cat _ion ~eferred t o this neoplasm as •:conge_nital Hilms ' t umor" but ther e 1·1as ah1ays t he observatiqn that the prognosis 1·1as exce~ lent. An abdomi nal ~ass in the neonate is the characteristic presentation and of course , the tumor should be di~tinguished from t he other causes of masses in this age, i.e., multicystic dysplasia of t he kidney, congenita 1 h_vdronephrosi s, ovai-i an cyst , hydroco 1 po.~ to mention some of the more common. The mesoblastic nephroma has also been associated with polyhydramni os . Congenital anor,1a lies are rar_e ly encount.ered with these t umors. . ' . The ri ght kidney is more commonly af fected than the l eft and all cases to dat e have been unilateral (a distinction from mass'ive bilateral nephroblastomatosis). The~e is virtual t otal repl acement 'of the renal parenchyma oy a solid mass l'lhich has the surface characteristics of the uterine leiomyoma. Cysts may recur and in fact, a unilateral multi~ocular cyst (variant, cystic ne9hroma) may contain a mesobl astic nephroma. The hi stology is relatively monotonous, consi sting of

RENAL TUi'IORS IN CHlLDREN < ONE. YEAR OLo·"

"Classic'' Nephroblastoma

Hesoblustic Nephroma Rhabdomyomatous Nephrobl a stoma · Cystic Nephroma Nonomor!)hous Tubular NephroblastoiJ!a "Rhabdoid" Hil ms' Tumor "Cl ear Cell" ·Hil ms ' Tumor

12 (7 -12 m/ o )

8 . · 6. l]-12 m/o)"

2 (2, 7 m/o) 2 (11, 12 m/o) 1 (1!-; m/o ) 1 (9 d;o)

*Ugarte, Gonzalez-Crussi and Hsueh (Unpubl ished, 1980) .

.\

B

closely packed spindle shaped cells t·tith a fascicular or lihOrled pattern.

Hitotic activity may be quite abundant diffusely or focally. Oys!Jlastic nests

of cartila~e and rare dys!llastic tubules are other findiQgs in 10-:15!:: of cases.

Sections through the renal hilum may demonstrate the presence of extrarenal tumors.

This finding does not have any adverse prognostic implications.

As though Beckt~ith 's proohesy l·las fulfil l ed 1·1hen he edi t oral ized about the

mesoblastic nephroma in 1974 as representi n~ "nrey zone" lesions of indetermi nan t

biologi cal s i gn'ificance, Gonzales-Crussi et a l. have reported a "cellul ar variant"

of mesob 1 ast i c n'erhroma ~1h i ch rnetastas'·i zed to the 1 ung. Their cas~ was reported

i n a child beyond 3 months of age and had mul tiole foci of necrosis. In general,

the mesoblastic ne!Jhroma s hould be vie1ed as a ben ign tumor but there does exist

the !JOtential, but rarely expressed. References

1. Beck1~ith JB: ~lesenchymal renal neo!Jlasms of. infancy revisited. J Pediatr Surg 9 :803, 1974.

2. · Bol ande RP: Congenital and· infant i le neoplasia of t he kidney. Lancet 2:1497, 1974 .

3. Bol ande RP: Congenital mesoblastic nepliroma of irif imcy. Perspect Pediatr Pathol -1 :227 , 1973.

4. Bolande RP et al. : Congenital mesoblastic nephroma of infancy. A report of eight

cases and the relationship to Hi l ms' tumor. Pediatrics .40;272 , 1967 .

5. Gonzalez-Crussi Fetal.: ~lalignant mesenchymal nephroma of infancy. Re!Jort

of a case with pulmonary metastases. Am J Surg Pathol 4:185, 1980.

Case 7. Kidney - bilateral Wi lms' tumor , rhabdomyomatous t ype.

This case is a perplexing one and again is a t est of our conceptual framework

of \-Jilms' tumor. The section discl oses essentially a monotypic neoplasm 1·1ith a

rhabdomyomatous appearance to be distinguished from one of the sarcomatous variants

of l·li 1 ms' tumor referred to as the "rhabdoid ( rhabdomyosarcomatous) type. The . . latter neoplasm is composed in part of polygonal cel l s with eosinophilic cytoplasm.

In the present case, the tumor cells are elongated and ·stra!)- like in .many instances ... ·

There l'las no evidence of cellular ana!Jlasia in my section . Although this patient

is considerably older than those reported by l·Jigger, it strikingly resembles the

"feta 1 rhabdomyoatous nephrob l a stoma". Penchansky and Ga·no re!Jorted some cases

as "rhabd?myosarcoma or' the kidne.)l" but these tumors are a mixture of entities

i ncluding the "clear cell sarcoma". There are some provocative comoarisons with

. 10

Pulmonary interstitial emphysema may occur as a primary di sorder in an

apparen t ly hea lthy fullterm infant or more commonly in a premature baby who devel ops

hyalin membrane di sease. In the latter instance , t he interstitia l emphysema

is a complication of respi rator assistance .

Ref erences

1. Hislop A, et al.: The l ungs in congenital bilateral renal agenes i s and dys­

plasia. Arch Dis £hild 54:32, . 1979. 2. Kissane Jt1: The morphology of renal cystic disease. cystic Diseases Of t he·

Kidney. Gardner KD, Jr (editor}, Ne~1 York, John Hiley & Sons , 1976. p. 31.

. 3. Uberman t·1M et al.: Associat io11 betlveen pneumomediastinum and renal anomalies. ' Arch Dis Child 44:471, 1969. t

4. Potter EL: Normal and Abnormal Development of the Kidney. Chicago, Year

Book Medical Publishers Inc., 1972, p. 209. 5. Rener~ HA et al.: Obstructive urologic malformations of the fetus and infant­

relation to neonatal pneumomediastinum and pneumothorax. (air- block}.

Radiology 105:97, 1972. 6. Stocker JT et al.: Cystic and congenital lung disease in the ne\~born.

Perspect Pediatr Pathol 4:93, 1978.

Case 9. Soft tissue, orbit a 1 - embryona 1 rhabdomyosarc.oma.

The polypoid tumor from the orbit rather than eye of this child is a highly

mali gnant neoplasm composed of small hyperchromatic cells 1~ith delicate eosinophilfc

cytoplasm. A search for cytoplasmic cross-stri ations yields ·in my experience

almost routinely negative results . It has become the accepted convention in most

cases of .embryonal rliabdomyosarcoma t hat cross-stri ations are not required ·for the

di agnosis. l~ith the concentration of tumor cells beneath the surface eoithelium,

thi s tunior qualifies as a "sarconia botryoides" 1·1hich is a gross pattern of growth

in those embryonal rhabdomyosarcomas extending into a ho.llow or potentially hollm~

spaces. As far as I am a1~are, there is no prognostic significance.

In a collected review of 255 rha-bdomyosarcomas in the head and neck regi.on

in children, the orbit and periorbital l ocation were the commones t co)ilbined .sites

(28o/,} follo1·1ed liy t he nasopharynx-nose (19%} and middl e ear (13%}. The resu) t s

of the Tntergroup Rhabdomyosarcoma Study ind i cat e that those tumors occurring

i n a parameningeal site such as the orbit have a _ si gnificant complication of

· CNS spread. These chi l dren are thus treat ed in a f ashion similar to acute leukemia

in childhood, receiving CNS prophylaxis.

9

h1o cases reported by Hughson et al . of bil ateral \·li l ms' t umor in \•Jhich one of

the t umors was a cytodifferentiated neoplasm. One of these cases had vtrtua7ly

identi c'a l rhabdomyomatous di f ferenti at ion. Myogenes i s in any !~i 1 ms ' tumor is

not an uncommon finding. It is present i n 10-15% of cases and t here is no

prognost ic signifi cance to its presence . Another interesting observation rel evant

to this case is the apparent maturational effect that i rradiation has upon t-lilms' tumor.

References

l. Bannayan GA et a 1. : Effect of i rradi ati on on the maturation of Ui lms' tumor. Cancer 27:812, 1971 .

2. Bech1ith JB and Palmer NF: Hi stopatho 1 ogy and prognosis of Hilms • tumor.

Results from the First National Hilms' Tumor Study. Cancer 41:1937, 1978.

3. Hughson MD et al. : Cytodifferentia ted renal tumors occurring with Hilms'

tumors of the opposite kidney. Report of t 1·10 cases. Am J Cl in Pathol

66 : 376, 1976.

4. t-lerten OF et al. : H.ilms ' tumor in adolescence. Cancer 37:1532, 1976.

5. Penthansky l and Gal l o G: Rha bdomyosarcoma of the kidney in children.

Cancer 44:285, 1979.

6. Trembl ay ~1 : Ultrastructure of a ~! ilms' tumour and myogenesis. J Pathol

105:269, 1971. 7. \•Jigger HJ: Fetal rhabdomyomatous nephrobl astoma ~ a variant of· tHlms ' tumor.

Hum Pathol 7:613, 1976.

Case 8 . Bilateral multicystic renal dysplasia (Potter's type 4) and pulmonary interstital emphysema.

The failure of the kidneys to develop normal ly has as this case illustrates

an ef fect upon the lungs. Because of the oligohydiamnios and possibly other

factors, there is marked reduction in 1 ung volume and in t he number of ai n-1ay

generations . These children come into the 1~orld 1~ith. hypoplasia and attendant /

compl ·icati ons. The hypoplastic lungs are capable of some expansion but the 1 imits

are quickly reached and rupture occur resul ting in pulmonary intersti tial emphysema,

pneumothorax and pneumomediastinum. The secti ons of the ·lung demonstrate the

elongated spaces i n the interlobar septa r epresenting the interstitial emphysema. Hhe

t he i nter stitial emP.hysema persists,a giant cell reaction to the ai r occurs in these

spaces. Bi l ateral renal dysplasia, renal agenesis and the othe r renal anomalies

associ ated Vlith the "Potter 's faci es" may have t hese complicai:i'ons.

10 .

P.ulmonary interstitial emphysema may occur as a primary d·isorder ·io un

apparentl y healthy f ull t erm inf ant or more commonly in a !)rematur.e baby v:ho dev~lop.s

.hyalin membran_e di sease. In t he lat~er instance, the interst i tii).l emphysema

i s a complicat ion of r es pi rator assistance .

Refere.nces

·1. · Hislop A, et al.: The l ungs i n congenital bilateral renal agenesis and· dys­. pTasia. Arch Dis Child' 54 :32, · ; ·979:"

2. ·- Kissane JN: The morphology_ of renal cyst.is :dise~se. Cy5.tic Di5eases of -the

- K·idney. Gardner KD, J r (edi tor ), New York, J ohn ~Iil ey & Sons, 1976. p . 31 .

· -3. Liberman t1M e! ·al.:' ASS!;)Ciatiojl bef1~eeri pneumomedh~'t;iJUm dl]d rena l ·anomal i es.

Arch Dis Child 44:471 , 1969. .,

4·. .Potter EL: Normal ·and "Abnormal Devel opment of t he Kidney. · Chicago·, Year .. .

Boo~ ~1edi_cal . Pub1ishe;s Inc., 1972, p. 2~9.

5. Renert HA et al. ~ Obstructive urol ogic malformations of the fetus and infant­

relation t o. ne-onat al pneumomediast i num. and pneumothorax (ajr- block.) .

Rad·iology 105 : 97 , 1972.

6. Stocker JT et a.l. : Cystic and congenital lung d·isease in the ne1~born . ..

Perspect Pediatr Pathol 4:93, 1978 :

Case 9. Soft tissue, orbital - embryonal rhal:ldomyosarcoma.

The polypoid t umor from the orbi t rather than eye of this child is- a h-i ghl.y

mal ignant neoplasm composed· ·of sma 11 liyperchromati'c cei 1 s ~~·i th del i cate eos i nop'hil i c

Gytopf asm. ti searc.h_ for cytoplasmic -cross--striati ons yiel ds in my experience

almost rout i nel y negat ive results . It has becomS'> the accepted conventtol'l in most . . . . . ca.ses o.f embryonal rliabdomyosar-coma that cross- stri·ations are not requ i'red· f or the··

diagnos is. ~~ i th the conceritratioi1 a·~ tumor cel l s beneath the surface . epithe 1 i urn '

th i s tumo.r gua 1 i fi es as a "sarcoma botryoi des" which is a gross p·attern of growth

i n those embryona 1 rhab.dom.vos<,~rcoma s extending into a ho 11 ow o·r potentially ho 11 O\v

spaces. As far as r· am aware, there is no prognostic signifi cance.

In ·a ~;;ollected revielv of 255 rhab.domyosarc.oma-s in the .head and neck region . .

in chi 1 dren, the· or bit and periorblta 1 1 ocati on were t he commonest combined sites

(28%) follo~ted by the nasopharynx-nose (19%) and mi ddle ear (13:~ ). The results

of the Intergroup Rhabdomyosarcoma Study i nd icate t hat t hose t umors occurring

in a parameningeal si t e such as the orbi t have a significant complication of

CNS -soread. These chil dren are thus t reated i n a f ashion similar to acute leukemia

in chil dhood , recei virrg CNS r rophyl axis .

11

Among tumors of the orbit in childhood, the 1·habdomyosarcoma constitutes

approximately one-fourth in the experi·ence of some series . There has been a

dramatic rever.s.a 1 i n the. rrevious di srna 1 prognosis with the combi nat i ol') of

i rradiation and chemo thera9y.

References

1. Dehner LP and Chen TKT :

III. A clinicopathologi c

104: 399' 1'978.

Primary tumors of the external and middle ear.

study of embryonal rhabdomyosar coma. Arch Otol ar_vngol

2. Gonzal ez-Crussi F and Bl ac k-Schaf f er S: Rhabdomyosarcoma of i nfa ncy and

childhood. Probl ems of morpholog.ic c l <;~ssif i cation . Am J Sur9 .Pathol 3:

157 ' 1979. 3. Iliff HJ ancrGreen HR : . Or bi tal t umors in children. Ocul a r and Adnexal

Tumors , J akobi ec FA (editor ) , Birmingham, Aesculapius Publishing Co .,

1978' p . 669.

4. Knol•tl es OM I I et al.: Opht halmic stria ted· muscl e neopl asms. Surv·-Opht-halmol

21 : 219, 1976.

5. Schuller OE et al . . : Childhood r habdomyosarcoma of the head and neck.

Arch Otolaryngol 105:689 , 1979.

6. Tefft t1 et al..: Incidence of meningeal involvement of r habdomyosarcoma of -

t he head and neck in children. A report of the Intergroup Rhabdomyosarcoma

Study ( I RS) . Cancer 42 :253 , 1978.

Case 10 . Granul.omatous i nf l ammat ioll containing phycomyces consi stent with mucor.

· Every si minar needs an i nexp 1 icabJ e sha9gy dog case and I s uppose that this

one apt l y qualifies. A mediastinal mass in a chi l d of this · age shoul d be ·a ma1 19nant

lymphoma, l ymphobl as t ic (T-cell.) ,. nodular sclerosinl) Ho-dgkin ' s disease or a germ

cell tumor if 1'/e presume an anter ior localization. and if possible , then a neurogenic

tumor, either a 9angl ioneurobl astoma or ~anglioneuroma. As i t t urns out , 1t is . . . . none of t he above but r ather necr.otizing granul omatous infl all]l!lation. There 1·1as

no evidence of a vas~uliti's and I make this co11'1llent sin~e· the se 9t:anulomas are

remi ni scent of Hegener' s granulomatos is. A chi ld f rom the 1·1ississippi Valley

should have histopl asmosis after ,of course tubercul osi~ has been ruled out. Special

stains, ho~tever, revea 1 ed irregular broad nonsep ta ted organisms consistent ~,; th the

Phycomyc~s group of fungi. There is no history that thi s child is immunodefici ent

nor has a history of di abetes mell itus. He are thus left 11i t h t hi s very peculiar

cl i ni cal s i t uation but one f rom the OIJerative desci-i9tion that could spell furt her

12

diff i cul t ies for t his child . The concern is the !JOSSible development of

scl erosing (fibrous) mediastini t i.s. · This is an ·infl <:urunatory .and desmoplastic

r.eact"ion in the_ rr:_edi <! stinum t hat insidiously soreads produ~ing r espirato·r.v

insuf f ici ency ana even th~ surer·ior vena caval syndrome. Inter·est i [!gly, this

complication occurs rnore often ·in young ind i vidt.:al s ~1ith histoplasmosis .

References

·1. Dines DE et a1 .: ~led i astina l granul oma and f ibrosing mediast 1n"itis . · Chest

75;320, ~979 .. 2. Fill er RM et a1.: 11ediasti nal masses in infants and children. Pediatr Cl i n

North Am 26 :677 , 1979. · -... 3. Pokorny HJ et al .: r•lediastinal masses in infants and chi ldre n. J Thorac:~

C.ardi ovasc Surg 68:869, 1974. · · . ..

4. \·lieder S and Rabinowitz JG : Fibrous mediastinitis: a late marrifestat;i.on_ .

of mediastinal histoplasmosis. Radiology 125:305, 1977 .

Case 11. Soft tissue - small blue cell tumor (? soft tissue equivalent of Ev;ing's sarcoma ) .

The mental exenise [)osed by this . essentially monotonous small cell neoplasm

of the soft tiss.ues ' isa not uncommon one for those of us 1·iho see .any number of

pediatric tumors. A~ extremely important pi ece of ini'ormaHon is whether there

i s bony f nvQlvement since osseoiJS E1•1i ng's sarcoma rnus1; be ru1_ed out at :the onset.

The most recent "f ly· i n the ointment" is the report of an "·osteosar coma 1·1ith

small cell~simulating E1·1ing's tumor". If 1·1e· assume the. localizati on to the

soft tissues , the standard differential diagnosis fs rhabdomyosarcoma, malignant

lymphoma and very unl·ike1y under any cirGumstances, a metastasis from a neuro-­

blastoma. The l ess conventiona l diagnoses are the so-ft tissue equival ent of

Ewing's sarcom~a and_ a periphera l mal i gnant neuroepi,thel ial tumor similar to tho~e

which I.\ skin et a l. reported in t he chest wa 11. The. appearance and arrangement

of the tumor cel l s are not right for rhabdomyosarcoma especially·an alveolar

rhabdomyosarcoma \·lith this patient ' s age and pri mary site. I did not think that

mal ign.ant lymphoma fit either so,therefore, I am left vtith some hi ghl y undif fer­

entiated neoplasm of presumed mesenchyma 1 deri vati o.n. It has become fashi onab 1 e

to refer to t hese tumors as "soft tissue' E1·1ing ' s s.arcom·a" but Angervall and

Enzi nger in t heir original [)Ublication des i qna ted t his neoplasm(s) o.ne "resembling

E11ing's sarcoma". G.i llesoie et ·al . have examined three cases by electron microscopy

1·1h i ch thev concluded have distinctive features. In terms of clinical behavi01· . -

13

and prognosis, the only information available is that of 26 cases froM the

Intergroup Rhabdomyosarcoma Study. These 26 cases represented Sr. of the . tumor

revie1~ed i n the IRS . From a11 indi cat ions , t here are more 'I.imilari t i es '.·lith

rhabdomyosarcoma t han E1·1i ng ' s sarcoma .

References

1. Angervall L and Enzinger F11: Extraskeletal neo!)l asm resembling E11i ng ' s

sarcoma. Cancer 36 :240, 1975 .

2. Aski n FB et al. : ~1ali gnan t small cel l t umor of t he thoracooulmonary reg ion

in chi l dhood. A disti nctive clinicopat hologic enti ty of uncertai n histo­

genesi s. Cancer 43 :2438 , 1979.

3. Dehner LP: Soft t i ssue sarcomas of childhood . The differ ential diagnostic

dilemma of t he "small bl ue cell" . Nat l Cancer Inst Monogr. Vol. 57, 1980.

4 . Gillespi e JJ et al.: Extraskel etal Ewing ' s sarcoma. Hi stologic and

ul trastructural observations i n t hree cases . Am J Surg Pathol 3: 99, 1979 .

5. Sim FH et al.: Osteosarcoma ~1ith small cells simul ating E~1ing 's tumor.

J Bone Joint Surg (Am) 61:207, 1979.

6. Soule EH et al.: ·Extraskel etal E1·1i ng' s sar coma. A prel iminary revie1~ of

26 cases encountered in t he Intergroup Rhabdomyosarcoma St udy. Cancer 42:

240, 1975.

Case 12. Skin - Spindl e-epi t hel ioid nevus (Spitz nevus).

Multi pl e blocks v1ere utili zed for the seminar so t hat some variation exi sts

i n t he hi stopathol ogi c features. · One of the sections ·discloses t hecques of sp·indl e

mel anocytes at the derma-epidermal junction. The cel ls a re rel at ively uniform

and t here i s no evi d~nce of atypia or mitot ic acti vi t y . There i s a l ight dustinq

of the cells by mel anin . In contrast , the other section reveal s small nes ts

and even isolated cell s in t he de rmi s Nith predominantly epithelioid characte r -

i stics. Some ce ll ul ar atypia i s noted but mi t ot·ic activity i s absent. Quite f rankly ,

if t he l esi on had occurred in an adult , r \~aul d have been t empted to diagnose it

as a malignant melanoma.

As you are aware, t he Spitz nevus occurs predominantly in the first decade

of life as a sol itary, pi nki sh t an, firm nodul e in t he head and neck regi on,

trunk or 10\•Jer extremities. Ther e are infrequent exampl es of mult ipl e l es 'ions

devel opi ng in an eruptive fa shion. Oesoite t he many admon'itions in the literature ,

the spindle-epithel ioid nevus is still mi s interpreted as mal ignant melanoma.

In various retrospective st udies , only 4-5% of putative mel anomas in children

have been the real thing .

t~ost Spitz nevi are l ess than 6 mm. in di ameter and the m<Jjority of l esions

are amel anotic. The so indl e patter n ~redominates i n 70-90)~ of cases and junctional­

dermal component is typically present. \·J hen the spindle cells arc prevalent,

they are uniform with few mitoses. Pi gmentation is usually absent. Hultinuc­

leated giant nevus cel ls and so-called myobl astoid cells are ot her findings .

The nuclei of the mononuclear nevus cells are l urge and t he nuc l eoli are

prominent. Epitheliomatous hyperpl asia of the ep ide rmis, vascul ar ectasia ir1 the

oapillary dermis, chronic inflalli'1ation and dermal fibrosis are other associated

features .

References ..

1. Ai nsworth A~l et a l.: Ne'l anocytic nevus, mel anocytomas, mel anocytic dysplas ias,

and uncafTl!lon forms of me 1 a noma. In Human ~Ia 1 i gnant ~lel anoma. Clark t·IH Jr,

Goldman LI and Nastrange 1 o ~~ (editors), Grune & Stratton, Ne1~ York , 1979,

p. 167.

2. Boodie AH Jr et al .: ~ialignant mel anoma in chil dren and young adults:

effect of di agnostic cri teria on s taging and end results . South t·1ed J

71:1074, 1978 .

3. Halec E et a1.: Re- appraisal of malignant melanoma diagnosis in the Swedish

Cancer Registry. Acta Path Microbial Scand (A) 85 :707 , 1977.

4. Okun t•IR: ~1e l anoma resembling spi ndle and epithelioid cell nevus . Repor t

of three cases . Arch Dermatol 115:1416, 1979.

5. Paniago-Pere ira C et al: Nevus of large spindle and/or epithel ioid cell s

(Spitz's nevus) . Arch Dermato1 114:1811, 1978.

6. Weedon D et al. :. Spindle and epithelioid cel l nevi in children and adults.

A revie1~ of 211 cases of the Spitz nevus. Cancer 40:217, 1977.