HUMAN PATHOLOGY

Volume 23, No. 1 (January

1992)

Patients with AIDS are predisposed to develop neoplasms as a result of a complex interaction of factors. Defective host ininiunosurveillance, presence of multiple co-infecting viral and nonviral organisms, unregulated polyclonal B-cell expansion, and abnormal regulation of various growth factors and cytokines’” are but a few of these promoting factors. Human immunodeficiency virus may possibly have a direct oncogenic role”’ or act indirectly through stimulation of growth factor elaboration by HIV-infected T cells.” In addition, the possible tumorigenic role of antiretroviral chemotherapy in treated patients must also be included as a cancer-predisposing facto1 for AIDS patients. Previous reports of neoplasms of smooth muscle origin in children with AIDS have detailed the variation in histologic aggressiveness from benign to overtly malignant in these tumors.’ It has also been suggested that there is an increasing incidence of smooth muscle neoplasms in HIV-infected children.7 In that regard, the present case represents the eighth smooth muscle tumor occurring in a pediatric p”tient with AIDS to be reported in the literature and is the first case report of hepatic leioni)osarcolna in a child with AIDS. In contrast to malignant lymphomas, smooth muscle tumors con~monly have not been associated with other causes of immurloclehcierlcy, including iInmunosuppressive medical therapy.L’L’ ” HoweveT-. it is possible that the reported increasing inc1denc.e of HI\’ infection in the pediatric. age group”’ will be accompanied by m increasing diagnosis of both benign md malignant smooth muscle tumors. including future cases of prinlar-y hepatic leiorlryosar-coIn;l. NOTED

AI)IlE:I)

IN I’KOOI;

‘l‘he patient died of I’mrittii pneumonia I I months after A conipl~re autopsy was perfornird. X0 residual tumoror recurrence was demonstrated. su~.gei?.

KEFE:KEN(:ES

MALIGNANT PERIPHERAL NERVE SHEATH TUMOR RETROPERITONEAL GANGLIONEUROMA: A CASE AND IMMUNOHISTOCHEMICAL STUDY

ARISING SPONTANEOUSLY FROM REPORT, REVIEW OF THE LITERATURE,

Malig~~anl IIr1-W shrath I~II~~OI~X 21-c 1111~~01~1n1011. and spontanec~us malignant tl.~ulsfol.l~l;~tioIl of ;t g~ulgliollrLII-oII1;1 is 1‘31~c. In the titrrarurr, wc fourd thrrtsimilar pwiord~ I-eportetf

of

;I

c-ases in which

sponLu1eous

S;~~~glioI~euro~i~;ic~cur-rrct.‘~“

previously

reportctl

c;wx

nlalignmlt

Thel-e

01‘ IIlatignant

;IIC wne

tr.;u~sfo7~~rialio~i ;ilso

thwv shrattl

ottw 11111101

arising in a pre-existir.lF g;i~iglio~icurom;r I.’ in which 1he tumors e\olced from a priniltlce r~curorc~tocler‘~l~;ll tumor after the\< patients rcceivetl radiation therapy. ;Zsidc l’r-oul tllcsc Cascb, iherr have hrc-11 ;I tiw brirf rrlcrcnce\ lo this 0ccuI~rellce.”

72

CASE STUDIES

FIGURE 1. (Top) Upper pole of left kidney placed by necrotic and hemorrhagic tumor. parenchyma invaded by spindle cell tumor; seen. (Hematoxylin-eosin stain: magnification

invaded and re(Bottom) Kidney no ganglion cells ‘c100.)

HUMAN PATHOLOGY

Volume 23. No. 1 (January

1992)

FIGURE 2. (Top) Clusters of ganglion cells present among benign spindle cells with wavy nuclei. (Hematoxylin-eosin stain; magnification X 100.) (Center) Ganglion cells intermingled with a highly cellular spindle cell component showing mitotic figures. (Hematoxylin-eosin stain; magnification X400.) (Bottom left) Neuronal processes stained with neurofilaments. (Avidin-biotin-immunoperoxidase stain; magnification X200.) (Bottom right) Spindle cells showing cytoplasmic and nuclear staining with S-100 protein. (Peroxidase-antiperoxidase stain; X200.)

l‘he ganglion cells showed intense cytoplasntic staining with tteurotl-specific enolase and neurofilaments. but were negative f’or S-l 00 protein. Neuronal processes also stained with ticurc6ilaments (Fig 2, bottom left). Many of the spindle cells of both benign and tttalignant components showed cytoplasmic and nuclear staining with S- 100 protein (Fig 2. bot-

ganglioneLtrom;t with inter-vetting areas of intertnediate grade ttmior itt the same tteoplasttt supports the belief that the malignant nerve sheath tuntot arose front the bcnigtt ganglioneurottta. In spilt of the absettcr of neitrohlastic~ ditierentiatic)n in this case as well as in the casts reported previously,‘~” the available data suggest that tnost g;iiiglionrut-01it~1s represent an ettd produrl of maturing iieurol,l;tst~)llia and g;iiigliotteurol~lastoma. In 1927, Gushing and Wolback were the first to describe the phettomrna of clinical regression and histologi,rlc,maturation of a neurohlastorn~t,!’ The presence of ganglioiieut-orl~a in the liver, spleen, lymph nodes. and hone itt some casts probably represents a maturing metastasizing gangliotirurohlastoma ot ~ieurohlastotiia.“‘~” Howrvcr. other investigators” believe that a g:;tiiglioneurottta does not necessarily at-ix by way of tnattrration of a pre-existing neurohlastottra, but map arise de ttovo. Kicci et 31’ reported two cases and Keller el aI” reportrd one case of malignant nerve sheath tumors arising from ganglioneurc,mas. These three patients had a history of radiatton therapy fat- treatment ofneurohl~tstoi~t~t or g;tligliolieut-ol,lastoma. Radiation therapy was used to induct trtaturation of thr primitive Il~uroectodermal tutttor. 111thrse casc’r, the devel-

tom right). The tumor cells were negative for desmin and muscle actin and were focally positive for vimentin. Only occasional cells were stained with I,eu-7.

We believe that this cast represents a malignant nerve sheath tumor arising from a retroperitoneal ganglioneuroma in an adult tnale. The immunohisto~hemical study confirmed that the spindle cell component of both benign and malignant aspects was schwannian in origin, being positive fix S-100 protein and focally positive for Leu-7. The ganglion cells were strongly positive for neuron-specific enolase and neurofilamerits. Although we could not document a sequential occurrence of the two neoplasms in our case, the presence of highgrade malignant tumor dispersed in the background of benign

74

CASE STUDIES

SEROUS PAPILLARY ARISING IN TESTIS: OBSERVATIONS

CYSTIC TUMOR OF BORDERLINE MALIGNANCY WITH FOCAL CARCINOMA CASE REPORT WITH IMMUNOHISTOCHEMICAL AND ULTRASTRUCTURAL

Asiotis” md Mtxistcl- et al.” N’e J~rescnt anothw exanlple of tllis grouJ) of’tuux~rs that may he c.lassitiCcl as uliillerian berou~ IxqAu~); c~ystadrnoma of tmrderlinc rnaligmnq with li~,11 tlrv~lopment of invasivr cauc~. The tumor has hrrn examined 1)~ light micr-osc-opv, illlrrlurlohistc,~hrnlistr~, and rlwtron mi-

(-t’osu’p\

(:.4SP: REI’OK’I The palient tcmsillrc~toiii\~ history of asyinl~tou~atic tvilargenwit of the right testis “siiic-c sew~A dwade~.” On adnlission. the right testis was l’ound to 1x2enlarged to about double the normal sizr ar~tl of hrm UHsistrim. The swelling was entireI\; paiulrus. I’aiiJxition failed to scJxuxte the totis Lr-ortl the epididw~ia. I~~~mwarkat~le laborator); findings resulted fi-oui urc&~, c011~putrd toIiwgrapt~v ol’lhc abrlo~r~ri~ and pelvis. hone xxi, at)domial.~l honography. C~nd hipedal lytl,J~ll;ln#ioa~;ipli~ I’or lymph node ~~wtxt;~st~. Tc.sticwIar sc,nograph) revcalcd a l;ir~c i~~tio~iro~~~ioi~s mx+s with foc,il liquefaction within the right htmiac rotunl. Again, the testis xrd cpididyulis could not be seJ)arat4 from eac+i other. On sut-gcy. Ihe right testis J)t-ovcd to h;~vc a fuw and, irl pi-l. 1)(m) cx~i~sislrrit~~. containing sot~lt vyrlic ;i~eab with Illilk> fluid. Thr tunica albuginea J~roved IO I,& unrelnarkahle without evidence of hydroccle or tumor- growtll. The coIltents 01‘ the right henlisc~rotuni (testis. rpidid~niis. and funic~ulus sJ’t.rmaticwS) wc’rc I-~~II~owI. -rllv patient I’0ll0wcd ,311 unvIlr;u-k;tt)lv po$tsurgic-A ~oursc. 011e wan. xflcr surgery. thew i\ 110 witltnc c 01’ tu1ii01 Ic’currenw 01 nlctaataw~. 01

75

Malignant peripheral nerve sheath tumor arising spontaneously from retroperitoneal ganglioneuroma: a case report, review of the literature, and immunohistochemical study.

We report a case of a malignant peripheral nerve sheath tumor arising from a retroperitoneal ganglioneuroma in an adult male. There was no known previ...
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