kaposi'slesions cases - from bmj and acp · kaposi's sarcomain lymphnodes: histological...

J. clin. Path., 1977, 30, 696-703

Kaposi's sarcoma in lymph nodes: histologicalstudy of lesions from 16 cases in MalawiK. M. O'CONNELL

From the Department of Morbid Anatomy, St Thomas's Hospital Medical School, London SE] 7EH,England

SUMMARY Biopsy of lymph nodes containing Kaposi's sarcoma from 16 patients showed that thetumour is identical in appearance with that of Kaposi's sarcoma of the skin, regardless of the ageof the patient or the mode of presentation. Spread of tumour along sinusoids throughout thelymph node was seen only in the cases of two children with generalised lymphadenopathy, butdiscrete deposits were present both in lymph nodes regional to skin lesions and in lymph nodesfrom patients who had presented with primary lymphadenopathy. The reaction of the remainder ofincompletely involved nodes was variable. No transition was seen between Kaposi's sarcoma andmalignant lymphoma.

Kaposi (1872) described the multiple pigmentedhaemorrhagic sarcoma of the skin, and later(Kaposi, 1894) he described a case with visceralinvolvement found at necropsy. Since then muchattention has been focused on skin lesions, which areby far the commonest form. But numerous studies inseveral parts of the world-particularly in Africa,where the disease is common-have shown thatlymph node and visceral lesions are by no means rare(Tedeschi et al., 1947; Reynolds et al., 1965; Slavinet al., 1969; Bhana et al., 1970). Unsuspected lymphnode and visceral lesions may be found at necropsyin patients who clinically appeared to have only skinlesions (Templeton, 1972). Occasionally lymph nodesdraining the site of a skin lesion are invaded bytumour (Bhana et al., 1970). In addition, a primarygeneralised lymphadenopathic type of Kaposi'ssarcoma is recognised. This is commonest in childrenand young adults and may be unassociated with anyskin lesions. It is usually rapidly fatal (Dutz andStout, 1960; Davies and Lothe, 1963; Slavin et al.,1970).This paper reviews the findings on biopsy of all

lymph nodes containing Kaposi's sarcoma receivedat St Thomas's Hospital from Malawi betweenJanuary 1969 and September 1975 inclusive. Thehistopathology of 159 cases of Kaposi's sarcoma ofthe skin and mucous membranes in patients inMalawi during the same period are discussed in aseparate paper (O'Connell, 1977).

Received for publication 21 February 1977

Material and methods

St Thomas's Hospital Medical School provides theonly histopathological service for Malawi, Thediagnosis has been made on sections of lymph podesstained with haematoxylin and eosin. Additionalsections of some nodes were stained with Perns' stainfor iron, Gordon and Sweet's reticulin stain, periodicacid Schiff reagent (PAS), phosphotungstic acidhaematoxylin (PTAH), Mallory's trichrome, andphloxine tartrazine. As with skin lesions, thetumours in lymph nodes were classified according totheir histological pattern into those with a mixed pat-tern, a predominantly spindle cell pattern, and ananaplastic group. When possible the distribution oftumour deposits within the lymph nodes wasexamined and also any abnormalities in the unaf-fected portions of nodes or in adjacent nodes.

Results

A total of 168 biopsy specimens showing Kaposi'ssarcoma from 159 patients were received during theperiod. Among them were 17 lymph nodes containingtumour from 16 patients (9 children and 7 adults).Two patients, cases 7 and 8, had both a skin and alymph node lesion. Some of the clinical and histo-logical features are summarised in Table 1.The tumours were classified into mixed type,

spindle cell predominant, or anaplastic types. Themixed type is composed of a pattern of interlacingbundles of spindle cells with abundant vascular slitscontaining red cells between adjacent tumour cells.

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Kaposi's sarcoma in lymph nodes

Table 1 Clinical and histological features in 16 cases of Kaposi's sarcoma in lymph nodes

Case no. Age Sex Histology type Extent ofnode involvement Clinicalfeatures(years)

1 Adult M Mixed Whole node Multiple lymphadenopathy2 3 M ,. ..3 20 M ,. ..4 2 F ,, Sinusoidal5 1 M ,, Whole node6 Adult M ,, Peripheral discrete deposits7 39 M ,, Discrete deposits Inguinal nodes + skin nodules8 60 M ,, Peripheral discrete deposits Scrotal nodules, chest wall nodules, and

inguinal nodes9 3 F ,, Sinusoidal spread Multiple nodes and skin nodules10 10 M ,, Whole node Multiple lymphadenopathy and skin

tumours11 4 M ,, ,, ,, Multiple lymphadenopathy12 9 M ,, Discrete deposits Inguinal lymphadenopathy13 Adult M ,, ,, ,, Multiple lymphadenopathy14 14 M ,, Whole node Cervical node15 6 F Spindle cell Discrete Inguinal lymphadenopathy

predominant16 Adult M Mixed Whole node Axillary lymph node and widespread skin

nodules

In the spindle cell predominant type vascular slits arevery much less evident and the cellular componentpredominates. In the anaplastic group the tumourcells themselves are much more pleomorphic, notalways spindle shaped, and the diagnosis is possibleonly if more typical areas are seen.

Fifteen of the 17 lymph nodes showed the mixedpattern. Vascular slits were plentiful and filled withred cells. Endothelial-lined channels and small bloodvessels within the tumour were often dilated so thatthe whole tumour appeared extremely vascular. Thelymph node tumours were often but not always morevascular than skin lesions of mixed pattern and thehistological features were essentially the same (Figs.1, 2). Mitoses were infrequent. Two lymph nodes,both from the same patient, a 6-year-old girl (case15), showed a predominantly spindle cell pattern withlittle vascularity. Mitoses, however, were infrequent.None of the lymph nodes examined showed an ana-plastic pattern. In cases 7 and 8 (a man of 39 and aman of 60 respectively) a biopsy of a skin nodule anda lymph node were done at different times. In bothcases the skin nodule and the lymph node showedtumour of identical mixed type appearance. The skintumours showed no histological evidence of aggres-sion or unusual mitotic activity.

In sections stained with haematoxylin and eosinclusters of eosinophilic hyaline bodies from about 1to 10 ,um in size have often been found in Kaposi'ssarcoma within spindle cells and in vascular slits.Their nature is uncertain. They resemble Russellbodies in their staining characteristics, though theyare found well away from any inflammatory infil-trate. They are particularly well seen with phos-photungstic acid haematoxylin and phloxine tar-

trazine, and have been noted in almost all skinlesions from Malawi. They were seen in sectionsstained with haematoxylin and eosin in 12 of the 17lymph nodes. Further sections were stained withPTAH and phloxine tartrazine and, altogether,hyaline bodies were found in 14 of the 17 nodes.

Perls' stain showed virtually no iron in theunaffected portions of the lymph nodes. Heavy irondeposition was often seen at the periphery oftumour deposits. Iron deposition within the tumourvaried from none at all in some areas to moderatelyheavy deposition in others. Reticulin stains con-firmed the observations of others (Lothe, 1963; Lee,1968) that a delicate reticulin framework surroundedalmost every cell. The amount of collagen demon-strated by Mallory's trichrome was extremelyvariable but some was present in almost all areas oftumour, often surrounding single cells and sometimeslining portions of vascular channels.

In eight biopsies the lymph nodes were totally oralmost totally replaced by tumour, so that it wasimpossible to comment on the pattern of involve-ment. In the remaining nine biopsies from eightpatients some lymphoid tissue remained. In twocases tumour was evenly distributed throughout thelymph node, predominantly along the sinusoids.Bands of tumour surrounded primary and secondarylymphoid follicles, which sometimes showed hyper-plastic germinal centres (Fig. 3). In the other sevenbiopsies tumour deposits were more localised. In twoof these (cases 6 and 8) the deposits were obviouslysituated peripherally (Fig. 4). Case 8 was an inguinalnode from a patient with a histologically provedscrotal lesion. The patient in case 6 presented withmultiple lymphadenopathy. Peripheral deposits of

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K. M. O'Connell

Fig. 1 Fig. 2

Fig. 1 Kaposi's sarcoma in lymph node. Note interlacing bundles of spindle cells separated by vascular slitscontaining red cells. Some are cut longitudinally and some transversely. Appearance is identical with that seen in skinlesions. (H and E x 170)Fig. 2 Kaposi's sarcoma in lymph node. Note extreme vascularity with many dilated, blood-filled channels.(H and E x 68)

Kaposi's sarcoma were seen and the remainder ofthe node was occupied by a malignant lymphoma ofdiffuse lymphoblastic type. In the remaining fivecases the tumour deposits in the node, though dis-crete, were too large to be able to say whether theirorigin was peripheral or not.

In some nodes even very large tumour depositswere nodular and very sharply demarcated from theremaining lymphoid tissue (Fig. 5). In other nodeslocalised deposits had begun to infiltrate adjacentareas of the node along the sinusoids. Both patternswere sometimes seen in the same lymph node.The appearances of unaffected portions of lymph

nodes are summarised in Table 2. Almost all tumourdeposits showed some degree of plasma cell pre-dominant inflammatory response at the margins,even when they occupied almost the whole node.Some nodes showed no reactive changes. Othersshowed follicular hyperplasia, sinus hyperplasia, orboth (Figs 6, 7).

Table 2 Reaction of unaffected portion of lymph nodesto tumour deposits

Reaction No. of cases

Follicular hyperplasia 5Sinus hyperplasia 2Plasma cell reaction around tumour 14No plasma cell reaction INo reaction in remaining node 2

Discussion

This review of biopsy findings confirms that theappearances of Kaposi's sarcoma in lymph nodes areidentical with those of Kaposi's sarcoma in the skin.As observed by others, the histology of a tumourfrom a child with generalised lymphadenopathy anda rapidly fatal outcome appears to be no more aggres-sive than the skin lesions of a middle-aged man with

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Fig. 4

Fig. 3 Predominantly sinusoidal spread of tumourssurrounding follicles with hyperplastic germinal centres.(H and E x 68)

Fig. 4 Peripherally situated deposists of tumour.(H and E x 8)

Fig. 5 Large, well circumscribed nodule of tumour inlymph node. (H and E x 4)

Fig. 3

Fig. 5

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K. M. O'Connell

Fig. 6 Lymph node with well circumscribed tumour.Pronouncedfollicular hyperplasia apparent in remainderof node. (H and E x 26)

I11011!|! ZiR SFig. 7 Wellcircumscribedtumour with sinus

i hyperplasia in:: x)uninvolvedportion

of node. (H andE x 22S5)

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Fig 8. Kaposi's sarcoma (arrowed) extending along sinusoids showing vascular reactivehyperplasia. It is difficult to know where tumour ends and reaction begins. (H and E x 100)

Fig. 9 Kaposi's sarcoma and malignant lymphoma. The malignant ymphoma in the lower left corner(bounded by arrowheads) and Kaposi's sarcoma above and to the right are distinct tumours. Darklystaining plasma cells (arrowed) are numerous it. the junctional zone. (H and E x 400)


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K. M. O'Connell

an indolent course over many years (Lothe, 1963;Slavin et al., 1969; Templeton, 1972).Bhana et al. (1970) found lymph node involvement

in 16 out of 48 consecutive cases of Kaposi's sarcomain adults. They suggested that the appearance ofincompletely involved nodes varied according towhether the gland was regional to skin disease orwhether it came from a patient with primary lymph-adenopathy. In the former, deposits were oftenadjacent to the subcapsular sinus, analogous tolymph node metastases from carcinomas By con-trast, in primary lymphadenopathy the tumourappeared to develop in the medulla of the node andexpand from within. The only lymph node in thisseries which was obviously secondary to a skinlesion was in case 8, where discrete peripheral depositswere seen. In the other lymph node with localisedperipheral tumour deposits the coexistence of malig-nant lymphoma made interpretation difficult. Thetwo cases with a uniform predominantly sinusoidaldistribution of tumour were both from children withmultiple lymphadenopathy. However, in some of thepatients presenting with primary lymphadenopathythere were discrete deposits of tumour in the lymphnode. These were sometimes single and sometimesmultiple.Lubin and Rywlin (1971) described changes in the

uninvolved portions of lymph nodes containingdeposits of Kaposi's sarcoma. The most strikingfeature was follicular hyperplasia with pronouncedhypervascularity of the germinal centres and someincrease in the vascularity of the interfollicular areas.The other striking feature was a heavy plasma cellinfiltration of the medullary cords. They describetransition between Kaposi's sarcoma and the hyper-vascular follicular hyperplasia. In the nodes fromMalawi hypervascularity of germinal centres waspresent where surrounding Kaposi's sarcoma wasjust beginning to invade the follicle. Otherwisereactive centres appeared no different from thoseseen in lymph nodes reacting to a wide variety ofstimuli. In general, the lymphoid follicles seemed tobe the areas most resistant to being engulfed bytumour. Two cases with sinus hyperplasia showedmarked hypervascularity of the sinusoids extendingsome distance from localised tumour deposits, and inthese it was difficult to be sure where tumour endedand reaction began (Fig. 8).The relationship of Kaposi's sarcoma to the

reticuloendothelial system is of continuing interest.Dorfell (1932) believed that Kaposi's sarcoma was aneoplasm of the reticuloendothelial system. Mildlymphocytosis or monocytosis and reports of atypicalmonocytes in the blood have been noted in somecases, but in most the leucocyte count has beennormal (Reynolds et al., 1965). Kaposi's sarcoma is

seen increasingly in association with reticulo-endothelial neoplasms, Hodgkin's disease, lympho-sarcoma, chronic lymphatic leukaemia, and myeloma(Reynolds et al., 1965). This association is much lesscommon in Africa, though a number of cases havebeen reported (Thijs, 1957; Uys and Bennet, 1958;Lothe, 1963; McKinney, 1967; Slavin et al., 1969).

Usually the two neoplasms have been quite dis-tinct. Lothe reported two cases from Uganda inwhich the tumours were present in adjacent tissues.In one case a lymph node contained both Kaposi'ssarcoma and lymphosarcoma. In the other theadrenal cortex showed adjacent Hodgkin's diseaseand Kaposi's sarcoma. Occasionally the tumourshave merged, particularly in lymphoma or leukaemicinfiltration of the skin (Sacks and Gray, 1945), and inone South African case the authors hinted at a transi-tion between Kaposi's sarcoma and Hodgkin'sdisease (Uys and Bennet, 1958). In the lymph node inthis series containing both Kaposi's sarcoma andlymphosarcoma spindle cells ran a short distance intothe lymphoma in some areas but the appearance didnot suggest any transition between the two. Plasmacells were numerous in the junctional zone (Fig. 9).

Recently, in an attempt to correlate immunologicaland histological findings, Warner and O'Loughlin(1975) put forward the interesting hypothesis thatKaposi's sarcoma is a by-product of an immuno-logical response to a primary tumour, probably alymphoma. They suggest that vascular proliferationis an integral part of the immunological response butthat it later becomes self-perpetuating. Theydraw an analogy with angioimmunoblastic lym-phadenopathy. Many of the tumour deposits in theMalawi lymph nodes were quite advanced and, un-like in the skin, adjacent early lesions were not seen.It was therefore difficult to be sure of the site ormode of origin of the tumour. However, clearly themode of spread of the tumour and the reaction ofthe remainder of the node were variable. Thereappeared to be no transition between lymphoma andKaposi's sarcoma, and only in the two lymph nodesshowing hypervascular sinus hyperplasia was thereany appearance of transition betweenreactivechangesand tumour.

I thank Professor H. Spencer, who founded andorganises the histological service for Malawi at StThomas's Hospital, Mr A. E. Clark and Mr J. S.Fenton for help with photography, and the technicalstaff of the Department of Morbid Anatomy forpreparing the sections. I am especially indebted toProfessor M. S. R. Hutt for advice and encourage-ment.ReferencesBhana, D., Templeton, A. C., Master, S. P., and Kyalwazi

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S. K. (1970). Kaposi sarcoma of lymph nodes. BritishJournal ofCancer, 24, 464-470.

Davies, J. N. P. and Lothe, F. (1963). Kaposi's sarcomain African children. In Symposium on Kaposi's Sarcoma,edited by L. V. Ackerman and J. F. Murray, pp. 81-86.Karger, Basle and New York.

Dorfell, J. (1932). Histogenesis of multiple idiopathichemorrhagic sarcoma of Kaposi. Archives of Derna-tology of Syphilology, 26, 608-634.

Dutz, W. and Stout, A. P. (1960). Kaposi's sarcoma ininfants and children. Cancer(Philadelphia), 13,684-694.

Kaposi, M. (1872). Idiopathisches multiples Pigment-sarkom der Haut. Archivfiur Dermatologie und Syphilis,4, 265-273.

Kaposi, M. (1894). Zur Nomenclatur des idiopathischenPigmentsarkoms Kaposi. Archiv fur Dermatologie undSyphilis, 29, 164.

Lee, F. D. (1968). A comparative study of Kaposi'ssarcoma and gianuloma pyogenicum in Uganda.Journal of Clinical Pathology, 21, 119-128.

Lothe, F. (1963). Kaposi's sarcoma in Uganda Africans.Acta Pathologica et Microbiologica Scandinavica,Supplement, 161.

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Reynolds, W. A., Winkelmann, R. K., and Soule, E. H.(1965). Kaposi's sarcoma: a clinicopathologic studywith particular reference to its relationship to thereticuloendothelial system. Medicine, 44, 419-443.

Sacks, W. and Gray, M. (1945). Kaposi's sarcoma andlymphatic leukemia. Report of a case with histologicevidence of two diseases in the same lesion. Archives ofDermatology and Syphilology, 51, 325-329.

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