aretroperitoneal tumour of the chemodectoma type · aretroperitoneal tumourofthe chemodectomatype...

J. clin. Path. (1964), 17, 444

A retroperitoneal tumour of the chemodectoma type

J. N. HARCOURT-WEBSTER

From the Department ofPathology, University of Edinburgh

SYNOPSIS A tumour of the chemodectoma type arising in the retroperitoneal space and projectingbetween the layers of the transverse mesocolon is reported. Attention is drawn to the lack ofcorrelation between the varied histological features and clinical findings in this group of tumoursand the inadvisability of forecasting behaviour.

The chemoreceptor cells of the carotid and aorticbodies and the glomus jugulare are sensitive tochanges in thepH and the 02 and CO2 tensions in theblood and produce reflex changes in respiratory andsympathetic activity (Schmidt and Comroe, 1940;Dripps and Comroe, 1944). Lent. C. Johnson(quoted by Smetana and Scott, 1951) describedcollections of histologically similar cells in theconnective tissue sheath of the femoral vessels.Similar foci have been described in the intra-abdominal and retroperitoneal tissues of rats andmice (Goormaghtigh, 1936; Hollinshead, 1942) butthey have not been conclusively demonstrated atthese sites in man.The infrequent tumours arising in the carotid and

aortic bodies and the glomus jugulare are wellrecognized (Lattes, 1950; LeCompte, 1951; Stewart,Ogilvie, and Sammon, 1956). Similar tumours arereported in the organs of Zuckerkandl (Cragg, 1934),the pancreas (Goodof and Lischer, 1943), the orbit(Lattes, 1950), the thigh (Randall and Walter, 1954),the extremities (Sirsat, 1954), the abdominal cavity(Arean and Ramirez De Arellano, 1956), and theretroperitoneal space (Zacks, 1958; Sessions andScott, 1960). The name of chemodectoma is oftenapplied to such histologically similar tumoursbelieved to arise from chemoreceptor tissues since itemphasizes the physiological properties of the tissueof origin without suggesting a sympathetic or otherspecific site of development (Mulligan, 1950). Forthose tumours, however, which arise at sites whereno chemoreceptor tissue has been satisfactorilydemonstrated the term non-chromaffin parangangli-oma may be preferred. The tumour now recorded isregarded as a further example of such a tumourarising in a very unusual site.

CASE REPORT

A married woman of 29 years, who had previously beenReceived for publication 10 January 1964.

in good health, presented with an eight-month historyof loss of appetite, flatulence, increasing listlessness andlethargy, and a painful swelling in the epigastrium. Afirm, slightly mobile, tender swelling was centred in theepigastrium slightly to the left of the midline. Thepulsatile character of the swelling was considered to betransmitted rather than direct. There was no lymphadeno-pathy or abnormality of the cardiovascular or respiratorysystems. Blood pressure readings before and afteroperation were within normal limits. A provisionaldiagnosis of a pancreatic cyst was supported radio-logically.At operation, a fairly mobile, solid tumour, 8 cm. in

diameter, was found projecting forwards from theposterior abdominal wall between the layers of thetransverse mesocolon. Numerous dilated veins transversedthe surface deep to the peritoneum. A plexus of similarveins invested the pedicle of the tumour which wasclosely adherent to both the aorta and the inferior venacava. Extensive haemorrhage from both series of veinsproved difficult to control and prevented removal ofthe tumour.The urinary catechol amine excretion measured after

operation was within normal limits.The patient was treated by x-ray therapy after which

there was a considerable improvement in her generalhealth. One year after the operation she was well and therewas no clinical or radiological evidence of metastases.

MACROSCOPIC

The biopsy taken at operation consisted of firm,pale brown tissue measuring 1 2 x 1 2 x 0 7 cm.The cut surface was moderately firm and faintlystriated. No capsular tissue was present.

HISTOLOGY

The tumour is composed of polygonal cells ofepithelioid type arranged in moderate sized clusterswithin a stroma formed by abundant elongatedvascular channels (Fig. 1). A reticulin preparationshows that in some areas reticular strands link the

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t Lj`L ._ s-SL _ A _AIII'MM': ~WRwF'FIG. 1. Generalpattern of tumour. Clusters or 'Zellballen' FIG. 2. Slender reticular strands link the vascularof epitheloid type cells with highly vascular stroma. channels and complete the stromal pattern. Gordon andHaematoxylin and eosin x 125. Sweets x 150.

FIG. 3 FIG. 4

FIG. 3. Pleomorphism of cells and their nuclei; bizarre cells including multinucleate forms. Centrally a collapsedsinusoid with tumour cells adjacent to endothelium. Haematoxylin and eosin x 400.FIG. 4. Large cell with a giant lobulated nucleus and conspicuous nucleoli. Haematoxylin and eosin x 575.

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J. N. Harcourt- Webster

TABLENON-CHROMAFFIN PARAGANGLIOMAS ARISING IN THE RETROPERITONEAL SPACE

Sex and Age Site of Tumour(s) BehaviourAuthor

Cragg (1934)

Goodof and Lischer (1943)

Smetana and Scott (1951)

Block et al. (1955)

Zacks (1958)

Sessions et al. (1959)

Sessions and Scott (1960)

F 30

M 47

M 23

F 27

On either side of abdominal aorta and lateral to origin ofinferior mesenteric arteryAnterior part body of pancreas. This case also included acarotid body tumour.Lumbo-sacral region with attachment to sacrum and rightpsoas muscleInvading muscle of lateral and posterior abdominal wall

No metastasis

No metastasis

Metastasis to lung andadrenalsMetastasis to lungs andliver

F 9 Over left psoas muscle and to left of great abdominal vessels No follow-upF 39 Slightly separated from upper pole of R. kidney No follow-upM 19 Right side promontory of sacrum; adherent to, but not invading No metastasis

R. coronary and internal iliac arteriesM 20 Above and behind second part of duodenum; adherent to inferior No metastasis

vena cava above renal veinsF 48 Along abdominal aorta and pelvic brim No metastasisM 17 Extending from diaphragm to aortic bifurcation and No metastasis

surrounding inferior vena cava and aorta distal to rightrenal vessels

M 17 Surrounding aorta and inferior vena cava from bifurcation No metastasisto renal vessels; thence posterior to aorta up to diaphragm

vessels (Fig. 2) though elsewhere these strands aredeficient.Though there is some variation, the cells generally

are large. Many are columnar or fusiform, and theirlong axes radiate from the walls of the vascularchannels. The cell membranes are well-defined andthere is abundant, finely granular, eosinophiliccytoplasm. Occasional cells show fuchsinophilia inthat the cytoplasm contains numerous, fine, orangegranules after staining with Masson's haematoxylin-Ponceau-fuchsin-light green; the chromaffin reac-tion is negative. Many nuclei are large, round,or oval and well-defined with a faint chromatinnetwork and one or two nucleoli (Fig. 3). A moderatenumber are hyperchromatic and there are scantymitotic figures. There are also a few small nuclei andoccasional very large forms, some of which arelobulated (Fig. 4); additionally, there are occasional,widely scattered giant cells with abundant cyto-plasm and variably sized and shaped nuclei up tothree or four in number.The vascular channels, many of which are

elongated and branched, are lined by a single layerof endothelial cells, some of which have a plumperswollen appearance. The larger vessels (? sinusoids)possess a thin cuff of connective tissue between theendothelium and the tumour cells. In this connectivetissue there are small numbers of fusiform cellsidentical with endothelial cells. No pigmentationwas noted and the Perls' prussian blue reaction wasnegative.

DISCUSSION

The non-chromaffin parangangliomas which havebeen reported as arising in the retroperitoneal space

are summarized in the table. A.P. Stout (1962,personal communication) includes an additionalgroup only one of which metastasized. Of these,three arose in the region of the organs of Zucker-kandl, two in the retroperitoneal tissue between thekidneys, two just below the kidneys, two at the rimof the pelvis or the sacral promontory, and one fromthe retroperitoneal tissue adherent to the duodenum.This latter example is closest in site to the tumourreported here. Though examples occur in all agegroups; the majority arise in the second to fourthdecades. There is no significant sex difference.The histological characteristics of the tumours

arising in the carotid body and glomus jugulare arewell known (Lattes, 1950; LeCompte, 1951). Thefeatures of this case and of those summarized areconsistent with those characters: nests or 'Zellballen',of pleomorphic large epithelioid type cells withina highly vascular reticular stroma, many cells beingclosely adjacent to thin-walled blood vessels. Lattesdescribed the occasional presence of haemosiderin.The origin of the abdominal tumours is uncertainowing to the lack of evidence for the constantpresence of chemoreceptor tissue in the abdomen ofman; further studies to determine the normaldistribution of this tissue within the abdomen arenecessary.

Similar neoplasms have been diagnosed asgranular cell myoblastomas, metastases from aprimary renal, hepatic or other carcinoma, lipo-sarcoma and angio-endothelioma (Kolodny, 1927;Smetana and Scott, 1951). Another group havingsimilar characteristics are the alveolar soft partsarcomas (Christopherson, Foote, and Stewart, 1952;MacFarlane and MacGregor, 1958); this groupmeta-stasizes particularly to the lungs. If the assumption

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is correct that the group of tumours, regarded aschemodectomas, are derived from chemoreceptortissue then it is possible that some of these other,histologically similar, tumours are derived fromsimilar tissue and should also be regarded aschemodectomas.

Regardless of site, the features of many of thesetumours in the chemodectoma group, particularlythe large bizarre cells, suggest malignancy but inmany of these cases neither the clinical findings northe subsequent behaviour confirms this. There isprobably no way, using cytological criteria, ofrecognizing malignancy in these tumours; thepresence of tumour cells within blood vessels is nota valid criterion of malignancy (LeCompte, 1951).Of those cases which have metastasized, the bones,lungs, lymph nodes, and liver are the usual siteswith occasional spread to the brain and adrenals.

It is of interest that the tumours of the chemo-dectoma group arising in sites below the diaphragmmetastasize more frequently than those sited abovethe diaphragn (Pettet, Woolner, and Judd, 1953);the subdiaphragmatic tumours, however, often onlymetastasize after a prolonged period of time thoughthere is an earlier tendency to local recurrence(Smetana and Scott, 1951).

I am indebted to Professor G. L. Montgomery forcriticisms and advice, to Mr. T. C. Dodds for the photo-

micrographs, and to Mr. E. L. Farquharson, underwhose care the patient was admitted, for permission topublish this case.

REFERENCES

Arean, V. M., and Ramirez De Arellano, G. A. R. (1956). Ann. Surg.,144, 133.

Block, M. A., Dockerty, M. B., and Waugh, J. M. (1955). Cancer(Philad.), 8, 97.

Christopherson, W. M., Foote, F. W. Jr., and Stewart, F. W. (1952).Ibid., 5, 100.

Cragg, R. W. (1934). Arch. Path., 18, 635.Dripps, R. D. Jr., and Comroe, J. H. Jr. (1944). Amer. J. med. Sci.,

208, 681.Goodof, I. I., and Lischer, C. E. (1943). Arch. Path., 35, 906.Goormaghtigh, N. (1936). J. Anat., 71, 77.Hollinshead, W. H. (1942). Anat. Rec., 84, 1.Kolodny, A. (1927). Surg. Gynec. Obstet., 44, suppl., 1, 163.Lattes, R. (1950). Cancer (Philad.), 3, 667.LeCompte, P. M. (1951). Tumors of the Carotid Body and Related

Structures, (Chemoreceptor System), p. 14. (Armed ForcesInstitute of Pathology. Atlas of Tumor Pathology, Sect. 4,Fasc. 16.) A.F.I.P., Washington.

MacFarlane, A., and MacGregor, A. R. (1958). Arch. Dis. Childh.,33, 55.

Mulligan, R. M. (1950). Amer. J. Path., 26, 680.Pettet, J. R., Woolner, L. B., and Judd, E. S. Jr. (1953). Ann. Surg.,

137, 465.Randall, K. J., and Walter, J. B. (1954). J. Path. Bact., 67, 69.Schmidt, C. F., and Comroe, J. H. Jr. (1940). Physiol. Rev., 20, 115.Sessions, R. T., McSwain, B., Carlson, R. I., and Scott, H. W. Jr.

(1959). Ann. Surg., 150, 808.-, and Scott, H. W. Jr. (1960). Amer. J. Surg., 99, 70.Sirsat, S. (1954). Indian J. med. Sci., 8, 800.Smetana, H. F., and Scott, W. F. Jr. (1951). Milit. Surg., 109, 330.Stewart, J. P., Ogilvie, R. F., and Sammon, J. D. (1956). J. Laryng.,

70, 196.Zacks, S. I. (1958). Amer. J. Path., 34, 293.

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aretroperitoneal tumour of the chemodectoma type · aretroperitoneal tumourofthe chemodectomatype...

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