L o c a l i s a t i o n g a s t r i q u e de s a r c o m e de Kapos i au cours de S ida

F. M U N I Z Z I , B. G A T F E S C H I * , G. PAGANO**, A. T E R R A G N A * * , M. C O N I O , H. A S T E . E n d o s c o p i c Service a n d Gastro in tes t inal Unit.

Is t i tu to N a z i o n a l e p e r la Ricerca su l Cancro- Genoa , Italy. *Depar tment o f Pa tho log ica l A n a t o m y .

Is t i tu to N a z i o n a l e p e r la Ricerca su l Cancro - Genoa , Italy. **Department o f In f ec t ious Diseases. Univers i ty o f G e n o a - Genoa , Italy.

Gastric localization of Kaposi's sarcoma in A.I.D.S.

RI~SUMI~

Les auteurs pr6sentent un cas de localisation gastrique de sarcome de Kaposi survenu chez un patient atteint de SIDA. Les aspects caract6ristiques des 16sions sont d6crits en soulignant les 616ments du diagnostic diff6rentiel avec les 16sions muqueuses et sous- muqueuses de l'estomac.

SUMMARY

The authors present a clinical case of gastric localization of Kaposi's sarcoma in a patient affected by AIDS. The typical characteristics of the lesion is reported. The differential diagnosis among other lesions involving the mucosal and submucosal structures of the stomach is stressed.

I N T R O D U C T I O N

Le sarcome de Kaposi consti tue une entit6 pathologique que l 'on peut re t rouver chez les malades atteint de SIDA, chez les malades soumis

une transplantat ion r6nale et chez t o u s l e s malades immuno-d6prim6s [2]. Une 6tude n6crop- sique r6cente a montr6 que son incidence au cours du S IDA est beaucoup plus 61ev6e que celle four- nie par le diagnostic p re -mor tem [3]. Dans cette 6tude, on a trouv6 le sarcome de Kaposi chez plus de 90 % des malades ; par cons6quent , sa pr6- sence possible doit 6tre envisag6e chez presque t o u s l e s malades atteint du SIDA.

La localisation du sarcome de Kaposi au niveau du tube digestif a 6t6 rappor t6e dans un pourcen- tage compris entre 58 % [3] et 77 % [1] des cas.

Le pr6sent travail rappor te un cas de syndrome de Kaposi ~ localisation gastrique d6tect6e par endoscopie et biopsie, chez un malade atteint de SIDA.

Cas c l in ique

Monsieur B.S. , 22 ans, toxicomane depuis l'gtge de 14 ans et homosexuel , s 'est aper~u en mai

1984, de l 'appari t ion d 'ad6nopathies g6n6ralis6es. Il souffrait en outre de pharyngites r6cidivantes avec for te fi6vre continue et t ranspirat ion abon- dante. Depuis quelques mois, il se plaignait 6gale- ment d 'un amaigrissement progressif, avec per te de poids de 13 kg. Une ant ibioth6rapie a 6t6 administr6e sans r6sultat.

En 1985, ~ la suite de douleurs abdominales diffuses, le malade est hospitalis6 dans un d6parte- ment chirurgical de l 'H6pital San Mar t ino de G6nes. Pendant cet te hospitalisation, on observe des nodules cutan6s multiples, violac6s, de 0 . 5 - 2 cm de diam6tre. D6s lots, le malade est transf6r6 dans le service des Maladies Infectieuses de l 'Uni- versit6 de G6nes, o~ l 'examen clinique r6v61e des ad6nopathies diffuses, une h6patom6galie, et une candidose orale. Les analyses h6matologiques et immunologiques mont ren t une leucop6nie, avec lymphop6nie , thrombop6nie , et une inversion du rappor t O K T 4 / O K T 8 .

De multiples biopsies des nodules cutan6s et une biopsie lymphonodale ont 6t6 prat iqu6es, qui posent le diagnostic de S IDA en phase aigu6 avec syndrome de Kaposi.

En raison d 'une dysphagie, une endoscopie du tube digestif sup6rieur a 6t6 effectu6e et mont re au niveau d e la paroi post6rieure du corps gastri-

Tir6s ~ part: Dr Hugo ASTE, Istituto Nazionale per la Ricerca sul Cancro, Viale Benedetto XV n~ 16132 Genova (Italy)

Mots-cl#s : 16sions gastriques polyp(aides, sarcome de Kaposi, SIDA.

Key-words : AIDS, Kaposi's sarcoma, polypoid gastric le- sions.

Acta Endoscopica Volume 16 - N ~ 4 - 1986 247

que, une 16sion polypoide, sessile, violac6e, non 6rod6e et recouverte par une muqueuse normale (Fig. 1). Trois biopsies sont pr61ev6es au niveau de cette 16sion.

T o u s l e s pr616vements ont 6t6 fix6s dans le formol neutre tamponn6 ~ 10 % et inclus dans la paraffine ; des fragments ganglionnaires ont 6t6 fix6s aussi en B.5. Outre les colorations ~ I 'H.E., une coloration de la r6ticuline et une coloration de Perls ont 6t6 r6alis6es.

Des sections histologiques de la peau montrent au niveau du derme, une augmentation marqu6e du nombre des espaces vasculaires qui apparais- sent dilat6s, irr6guli~rement branch6s, avec des parois minces parfois hyalinis6es, bord6es par des cellules endoth61iales atypiques, avec de rares mitoses et autour de ces cellules, un faible infiltrat inflammatoire. Ces aspects morphologiques ont permis le diagnostic de sarcome de Kaposi de la peau dans la phase en plaques (Fig 2) [2].

L'aspect typique de cette maladie a 6t6 retrouv6 l'examen histologique des fragments ganglion-

naires qui montre un effacement de l'architecture normale, une prolif6ration de petits vaisseaux comparables ~ ceux de la 16sion cutan6e, avec envahissement des tissus p6ri-ganglionnaires, ainsi que des foyers de hyalinisation avec infiltration lympho-plasmocytaire.

L'examen histopathologique de la 16sion gastri- que a montr6 dans le chorion, une prolif6ration de petits espaces vasculaires entour6s par des cellules fusiformes, en courts faisceaux d61imitant des espaces irr6guliers contenant des 6rythrocytes et des d6p6ts d'h6mosid6rine.

Ce tableau, malgr6 la quantit6 limit6e de tissu disponible due au pr616vement assez superficiel,

est compatible avec le diagnostic de sarcome de Kaposi (Fig. 3-4).

D I S C U S S I O N

D~s 1981, la litt6rature rapporte des cas de sarcome de Kaposi observ6s chez des malades homosexuels atteints de SIDA [1]. On doit aussi souligner que ce groupe de malades pr6sente une atteinte du syst~me gastro-ent6rique dans un pour- centage sup6rieur h 50 % [1] et que ces localisa- tions sont asymptomatiques dans un pourcentage 61ev6 de cas [4]. I1 est donc 6vident qu'il est utile, lors de manifestations cutan6es de sarcome de Kaposi, d 'explorer par voie endoscopique, le tube digestif sup6rieur et 6ventuellement le c61on, m6me en l'absence de sympt6mes, ce qui est le cas de notre malade [5].

La 16sion est caract6ristique soit du fait de sa couleur violac6e, soit en raison de sa forme, qui selon Nisar [4], peut 6tre: maculo-papulaire (A), polypoide (B), crat6riforme (C).

Dans notre cas, l'aspect est superposable au module B. Pourtant, /l notre avis, le diagnostic diff6rentiel doit prendre en consid6ration toutes les 16sions qui peuvent survenir au niveau de la sous-muqueuse, c'est-h-dire: un lymphome, un 16iomyome, un neurinome et une ectopie pancr6a- tique.

Cette discrimination nous incite ~ souligner l'importance de biopsies convenables permettant un diagnostic histologique correct.

Les auteurs remercient la "Fondation Maria Piaggio Casarsa" de Gdnes p o u r son assistance technique.

RI~FI~RENCES

l. FRIEDMAN S.L., WRIGHT T.L., ALTMAN D.F. - - Gastrointestinal Kaposi's sarcoma in patients with acquired immunodeficiency syndrome. Gastroenterology, 1985, 89, 102-108.

2. GOTTLIEB G.J., ACKERMAN A.B. - - Kaposi's sarcoma, an extensively disseminated form in young homosexual men. Hum. Pathol., 1982, 13, 882-892.

3. MOSKOWlTZ L.B., HENSLEY G.T., GOULD E.W., WEISS S.D. - - Frequency and anatomic distribution of

lymphadenopathic Kaposi's sarcoma in the acquired immu- nodeficiency syndrome. H u m Pathol., 1985, 16, 447-456.

4. NISAR A., NELSON R.S., GOLDSTEIN H.M., SINKO- VICS J.G. - - Kaposi's sarcoma of the stomach and duode- num : endoscopic and roentgenologic correlations. Gastroin- test. Endoscopy, 1975, 21, 4, 149-152.

5. PORT J.H., TRAUBE J., WINANS C.S. - - The visceral manifestations of Kaposi's sarcoma. Gastrointest. Endoscopy, 1982, 28, 3, 179-181

248 Volume 16 - N ~ 4 - 1986 Ac ta Endoscopica

Figure Lesion polypo'ide sessile du corps gastrique,

Polypoid sessile lesion of the gastric body.

Figure 2

Fragment de peau: dans le derme superficiel, augmentation du nom- bre des espaces vasculaires dilates et irr6guli~rement branch6s. Cet aspect histologique est typique du sarcome de Kaposi dans la phase

en plaques (H.E. x 6.3). Skin fragment showing in the superficial derma an increase of the number of vascular spaces appearing dilated and irregularly branched. This histological aspect is typical of Kaposi's sarcoma in the plaque

phase (H.E. x 6.3).

Figure 3 Biopsie gastrique endoscopique de sarcome de Kaposi. On observe de nombreux petits espaces vasculaires entour6,s par des cellules fusi-

formes. (H.E. x 25). Gastric endoscopic biopsy of Kaposi's sarcoma. One can observe the proliferation of the small vascular spaces surrounded by fusiform cells

(H.E. x 25).

Figure 4 Agrandissement photogrephique d'un detail de la Fig. 3.

Enlarged photograph of a detail related to Fig. 3.

INTRODUCTION

Kaposi's sarcoma constitutes a pathological entity that one can f ind in patients affected by AIDS, in patients who have undergone kidney transplants and in all those patients presenting an immuno- depressive picture [2]. A recent necroscopic study showed that the incidence during the course of A I D S is very much higher compared with that o f pre-mortem diagnoses [3]. In this study Kaposi's sarcoma was found in more than 90 % o f the

patients ; and consequently one can hypothesize its possible presence in nearly all patients affected by AIDS.

Localization o f Kaposi's sarcoma in the digestive tract has been reported in a percentage between 58 % [3] and 77 % [1] o f the cases.

The aim o f this work is the presentation o f a case o f Kaposi's sarcoma arising during the course of AIDS, with localization in the gastric tract detec- ted by means o f endoscopy and biopsy.

Acta Endoscopica Volume 16 - N ~ 4 - 1986 249

Clinical case

Mr. B.S., 22 years old, drug addict since the age of 14 and a homosexual, became aware in 1984 of a generalized adenopathy, accompanied by recur- rent pharyngitis, continuous high temperature and pronounced sweating. After some months the patient complained o f progressive weight loss, having lost around 13 kg. He was subjected to a course of antibiotics but without any result.

In 1985 the patient was taken into hospital, in the Surgical Department o f St. Martino Hospital, Genoa, following the onset o f diffuse abdominal pain. During his stay in hospital multiple violet- coloured skin nodules, from 0.5 - 2.0 cm in diame- ter, were found. He was therefore transferred to the Special Department for Infectious Diseases of the University of Genoa. On examination he was found to have diffused adenopathy, an enlarged liver and oral thrush. Subsequent blood tests together with immunological tests revealed leucopenia, thrombo- penia, lymphopenia and inversion of the ratio OKT4/ OKT8.

Multiple biopsies o f the skin nodules and a lym- phonodal biopsy were carried out, which allowed the diagnosis o f A I D S in the acute phase with Kaposi's sarcoma. The patient complained of dys- phagia and as a result o f this an endoscopy o f the upper digestive tract was carried out, showing at the level o f the upper wall o f the gastric tract a polypoid, sessile lesion. This was violet-coloured, non eroded and covered with normal mucous lining (Fig. 1). Three biopsies o f this lesion were carried out.

All the samples were fixed in a 10 % buffered neutral Formol solution and embedded in paraffin. Some lymphnodes fragments were also fixed in B.5. In addition, H.E. dye test, for the reticuline and Perls dye test were carried out. The histological sections of the skin showed the presence in the derma of a marked increased number of vascular spaces, which appeared to be dilated, irregularly branched, with thin walls sometimes hyalinized, bordered by atypical endothelial cells with rare mitosis and surrounded by a slight inflammatory infiltration. These morphological aspects allowed the diagnosis o f Kaposi's sarcoma of the skin in the plaque phase (Fig. 2) [2].

The characteristic aspect o f this disease was found on histological examination of the lym- phnodes fragments, which showed a cancelling out o f normal architecture, a proliferation of small ves- sels similar to those of the skin lesions invading the lymphnodes tissues, together with centres of hyalini- zation and lympho-plasmocitary infiltration.

The histopathological examination of the gastric lesion showed a proliferation o f the small vascular spaces in the chorium, surrounded by fusiform cells in short bundles, defining some irregular spaces containing some erythrocytes and deposits of hemo- siderine.

This picture, even if quantitatively modest owing to the fairly superficial samples, is compatible with the diagnosis o f Kaposi's sarcoma (Fig. 3, 4).

D I S C U S S I O N

Cases of Kaposi's sarcoma in homosexual patients affected by A I D S can be found in medical literature since 1981 [1]. It must also be underlined that this group of patients showed an affection of the gastro-enteric system in a percentage of more than 50 % [1] and that these localizations were asymptomatic in a high percentage of the cases [4]. It is therefore clear that it would be useful, where Kaposi's sarcoma of the skin is present, to carry out an endoscopic examination of the upper diges- tive tract and, should the occasion arise, also the colon, even in the absence o f symptoms, as in our case [5]. Typical characteristics of the lesion is its violet colour and its shape, which according to Nisar [4] could be either: maculo-papillary (A), polypoid (B), crateriform (C). In this particular case the aspect o f the lesion corresponded to type (B).

However, in our opinion one must take into consideration also a differential diagnosis among all the lesions which can originate in the sub-mucous stratum, that is : lymphoma, leiomyoma, neuri- noma and pancreatic ectopia.

For this reason it is necessary to underline the importance of appropriate biopsies with the aim of making a correct histological diagnosis.

250 Volume 16 - N ~ 4 - 1986 Acta Endoscopica