The original publication is available at http://www.samj.org.zaA 10th case of extramedullary plasmacytoma (EMP) of the colon is reported. The term 'primary isolated extramedullary plasmacytoma' is preferred to those terms previously used. A broad classification of plasmacytoma of the gastrointestinal tract is suggested and a management policy, based on this classification, is outlined.Publishers' versio

Allison, J. G.

Getaz, E. P.

Staples, W. G.

Stellenbosch University SUNScholar Repository

I1 Junie 1977 SA MEDIESE TYDSKRIFPrimary Isolated Extramedullary Plasmacytoma of theColonA Case ReportJ. G. ALUSON. E. P. GE'.TAZ. W. G. STAPLESSUMMARYA tenth case of extramedullary plasmacytoma (EMP) ofthe colon is reported. The term 'primary isolated extra-medullary plasmacytoma' is preferred to those terms pre-viously used. A broad classification of plasmacytoma ofthe gastro-intestinal tract is suggested and a managementpolicy, based on this classification, is outlined.5. rJfr. med. J., 51. 893 (1977).Plasmacytoma of the colon or rectum is an uncommonlesion. Only 9 cases have previously been reported in theEnglish literature. Of these. only 4, by virtue of adequatedocumentation. can be regarded as primary isolated plas-macytomas of the large bowel."" This implies that thereis no evidence of bone marrow plasmacytosis. BenceJones proteinuria, or radiological changes in bone.A tenth case of extramedullary plasmacytoma (EMP)ari ing from the large bowel is pres nted. it is the fifth-a e of primary isolated EM P reported as having occurredat this site. Of the previously reported colonic plasma·cytomas 5, by virtue of specific positive findings of gene-ralized plasma cell dyscrasia or the absence of documentedexclusion of such manifestation. cannot truly be classifiedas primary and isolated. The patient reported by Hamptonand Gandy.' in whom the rectum was involved. hadundergone resection of a previous plasmacytoma of thestomach. and the lesion cannot therefore be consideredpnmary.CASE REPORTA 61-year-old White man was admitted to the surgicalward for Billroth I gastrectomy for a gastric ulcer. diag-nosis of which was confirmed by barium meal examinationand fibre-optic gastroscopy. Results of investigations onadmission were as follows: haemoglobin 12, I g 100 ml.white cell count 9400,.,.1 and ESR 75 mm 1st h (Wester-gren). Electrolyte and acid base statL'S. and liver and renalfunction were normal. The chest radiograph was normal.Lymphoma Service, Tygerberg Hospital, and Departments ofSurgery and Haematology, Universit}, of StelJenbosch.ParowvalJei, CPJ. G. A.LLI 0:\. F.R.C.S.. F.C.S. (S.A.)E. P. GET.-\Z. .\l.B. CH.B.. .\l.R.C.p. (Pre ent aclclre s: Dept of'declical Oncolog\·. Hosn'ell Park ~Iemorial Institute. Buffalo.:\Y. USA)\\'. G. STAPLES. .\1.}.I£O. (HAD/. PATH.). F.F. PATH. (s ..~.)Dale received: II March 1977.Reprint requests 10: Dr W. G. Staples, Department of Haematology,UnlversilY of Slellenbosch Medical School. PO Box 63. Tygerberg. 750-RSA.and the ECG showed mild left ventricular strain. A po itiveoccult blood test on rectal examination was ascribed to higastric ulcer.On 3 March 1976, the abdomen was opened via an uppermidline incision. Routine examination of the abdominalviscera disclosed a mass in the di tal sigmoid olon.Since the colon had not been prepared. no resection waattempted. An enlarged lymph node of the inferior mesen·teric group was removed for histological examination.This was subsequently reported as having hown 're·active changes only'. A gastrectomy was not performed.After an uneventful recovery of the patient. a bari12menema examination (Fig. I) supported the suspicion of amalignant lesion of the sigmoid colon. beyond the reachof a rigid sigmoidoscope. Colonoscop~ was not availableto us.Fig. 1. Radiograph of barium enema examination, showingthe filling defect (arrowed) in the distal sigmoid colon.After adequate pharmacological and mechanical bowelpreparation. the patient underwent a sigmoid colectomyon 7 April, with primary end-to-end anastomosis. Post-operative recovery was uncomplicated.Histology of the lesion (Fig. 2) was reported on asfollows:'Histology IS that of a plasmacytoma, with sheet ofplasma cells, some of which are binucleated. Mitoticfigures are infrequent. Calcification of the tumour andsubmucosa is present. and there are iron pigment depositin the former. The tumour is well circumscribed, involvingthe mucosa and submucosa but also extending into themuscularis propria. Superficial ulceration and secondary94 SA MEDIC.-\L JOURNAL 1I June 1977acute inflammator~ changes are present. The tumour issurrounded by focal area of lymphocytic infiltrationand the regional lymph nodes show non-specific reaction.Total ex ision i presumed. a the limits of the specimenare through normal tissue. Scattered diverticula are pre-sent in the colon.'Fig. 2. Section of tumour showing sbeets of plasma cells,a few larger plasmab~asts, and a paucity of mitoticfigures (H and E x 400).In view of the diagno is. further investigations were per-formed. Repeat screens of Jiver and renal function werenormal. The serum uric acid level was within normallimits. There was no proteinuria. no Bence lones protein.and the serum albumin level was normal, with a normalglobulin pattern on electrophoresis. Bone marrow aspirateand trephine biopsy show~d no evidence of myeloma.Radiological skeletal survey was normal.Repeat gastroscopy on 15 April revealed that the gastriculcer had healed.DISCUSSIONDolin and Dewar' have classified neoplastic plasma cellproliferation as (i) multiple myeloma; (ii) solitary myelomaof bone; (iii) plasma cell leukaemia and (iv) EMP. Theystate that the EMPs do not exhibit the manifestations ofdisturbed protein metabolism. Demonstration of abnormalproteins (paraproteins) would therefore preclude the diag-nosis of primary isolated EMP. Others,"· however, be-lieve that produ tion of an M protein by the cells of ucha tumour is compatible with the diagnosis, provided thatdi appearance of the monoclonal peak follows completeresection.A further distingui hing feature is lymph node involve-ment. This is common in primary isolated EMP. but isseldom found in other foci of plasma cell neoplasia:'In a comprehensive review. Wiltshaw" has stressed theimportance of regarding primary isolated EM P a apathological entity separate from other plasma cell dys-crasias. This distinction has a bearing on the modalitiesof treatment and on prognosis.Approximately 10', of all EMPs are found in thegastro-intestinal tract.'·'·' but fewer than half of these maybe regarded as primary and isolated." Extramedullaryplasmacytoma of the bowel has been described as partof multiple myelomatosis.'· The majority of primaryisolated EMPs show evidence of malignancy. but subse-quent development of generalized disease is uncommon.'and should probably cast doubt upon the original diag-nosis.We feel that all primary isolated EMPs should be con-sidered malignant" and treated accordingly. Most authorsrecognize the role of local resection and irradiation, butchemotherapy is of dubious value.'" It should be con-sidered, however, when there are distant metastases·EMP, although solitary, but part of a generalizedplasma cell dyscrasia, should be treated locally on itsmerits. in addition to conventional aggressive treatment formyelomatosis.A rational systematized approach to the managementof EMP includes the following investigations: full bloodcount. peripheral smear. and estimation of erythrocytesedimentation rate; blood urea, serum creatinine, andcreatinine clearance; liver function studies, and serumprotein electrophoresis: immunodiffusion and immuno-electrophoresis; urine analysis for Bence lones protein;bilateral trephine bone marrow biopsy (repeated biopsiesat annual follow-up unless otherwise indicated) and radio-logical and isotopic skeletal survey.These parameters are assessed at the time of diag-nosis of EMP and at 6-monthly intervals after treatment.to exclude synchronous or metachronous plasma cell dys-crasia ..Treatment of gastro-intestinal EMP is based on itsclassification into either primary isolated EMP (and itstaging) or EMP associated with synchronous or ,meta-chronous generalized disease (Table I).TABLE I. CLASSIFICATION, STAGING AND TREATMENT OF GASTRO-INTESTINAL EXTRAMEDULLARY PLASMACYTOMAClassificationGroup AGroup BGroup CStagingStage I (localized disease)Stage 11 (lymph nodes or residual tumour)Stage III (distant disease)Synchronous generalized diseaseMetachronous generalized diseaseTreatmentResectionResection and radiotherapy·Resection, radiotherapy and chemotherapytResection if indicated, radiotherapy, andchemotherapyChemotherapy• Radiotherapy: local field and regional nodal irradiation.t Chemotherapy: full therapy as for multiple myeloma.II Junie 1977 s EDIE E TYDSKRIFGroup A constitute primary isolated EM P. This implieno stigmata of plasma cell dyscrasia. although evidenceof an M component which disappears after complete re-e'tion does not preclude this diagnosis. Infiltration ofadjacent tissue by the tumour, which is nevertheless re-sectable. still leaves the tumour in stage I. Histologicalevidence of incomplete resection warrants reoperation.jf feasible. It not, the tumour should be restaged as stage11. Distant metastases automatically indicate stage IIIdisease.Resection of a gastro-intestinal EMP associated withsynchronous disease (group B) should be performed if in-dicated. e.g. by intestinal obstruction or haemorrhage.The appearance of metachronous generalized disease(group C) is unusual. but may occur.' The original diag-nosis of primary isolated EMP should be reviewed. If theonly evidence of possible metachronou di ease i the re-appearance of a monoelonal peak. local recurren -e of thetumour must be excluded before chemotherapy is con-sidered.REFERENCE.-I. Nielsc:n. S. 'I.. Sch~nkcn. J. R. and Cawlt:y. L. P. (1971): Cancer.30. 261.1. Schwc:crs. C. A .. Shaw. 1\1. T .. Non.1qui ... t. R. E. ('t al. (1976):Ihid .. 37. 2220.3. \Ving.. E. J _. Pachick. J. amI HubbarJ. J. (1975): J. Amt.'r. Oleo.A;, .. 2J3 1298.-+. Hampton. J. 1\1. and Gandy. J. R. (19571: Ann.. urg .. 1...l5. '+15.5. Dolin. S. and Dc:war. J. P. (1950). Arncr. J. Pathul.. 32. '3.b. \Vi!u.haw. E. (1976); Medicine. 55. 217.7. Godard. J. E., Fox. J. E. and Levin,on. :'-1. 1. (I 3): J. digest.Dis.. 18. -08.hanna. K. D. and hriva"'la\. J. D. (I 01): Ardl. PathoL. 71. 229.9. Wiltshaw. E. (1971): Brit. med. 1.. 2. 32 .10. Hayes. D. \V.. Rennet. \V. A. and Hel.:k.. F. J. t1 .:':!): Arch. Palhol..53. 162.11. Stiff. R. H. and F<rraro. E. F. (I 72): 1. oral :'-f<J .. 27. 22.Infantile Cortical Hyperostosis (Caffey's Disease)A Case ReportD. J. FRIEDMA J_ M. ISDALE. S. JACOBSO S. E. LEVISUMMARYAn infant in hospital unexpectedly developed infantilecortical hyperostosis (Caffey's disease) while under-going treatment for an unrelated illness. The presentationof the disease was classic and there was markedthrombocytosis. The aetiological possibilities are dis-cussed.S. A fr. med. J., 51, 895 (1977).Caffey"s disease or infantile cortical hyperostosis wasdescribed by Caffey and Silverman in 1945. The diseaseis characterized by subperiosteal new-bone formationinvolving multiple bones and associated with tendernessand swelling of the overlying soft tissues. It is a raredisease with an unknown aetiology. An unusual featureof the case we wish to present is that it developed whilethe patient was in hospital undergoing treatment for aDepartments of Paediatrics and Radiology, TransvaalMemorial Hospital for Children and University of theWiiwatersrand, JohannesburgD. J. FRIEDMA!\". ::-I.B. B.CH.. D.C.H .. F.C.P. (S.A.)J. :\1. ISDALE. M.B. B.CH., ::-DIED. RAD.(D.). JACOBSO'\_ ::-I.B. B.CH.. D.C.H.. F.C.P. (s ..",.). E. LEVIN, M.B. B.CR., F.R.C.P.E.. D.C.I-!.Date received: 17 December 1976.different illness. Furthermore. it is apparent from thecircumstances surrounding the patient's illness that aninfective aetiology cannot be dismissed. The strikingfhrombocytosis that may occur during the course of theillness is also documented.CASE REPORTThe patient was a White female infant who was born athome on 9 March 1975. following a normal vertexdelivery. Her birth weight was 2.46 kg. No maternalillness was recorded during pregnancy and no problemarose during the neonatal period. At 3+ month of agethe patient developed a urinary tract infection and anexcretory urogram demonstrated an obstru tion of theleft pelvi-ureteric junction. She was referred to ourhospital for surgical correction of this anomaly. Onadmission her weight was recorded as 4. kg (3rd Bostonpercentile) and her length and head circumferencemeasurements were between the 50th and 75th percen-tiles respectively. There were no other iblings in thefamily.Examination revealed generalized hypotonia with somedelay in motor development. The re t of the examina-tion revealed no abnormality. An X-ray film of the chesttaken at this stage was normal (Fig. I). Before transferto our hospital the haemoglobin level wa .9 g 100 ml

Primary isolated extramedullary plasmacytoma of the colon: a case report

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Primary isolated extramedullary plasmacytoma of the colon: a case report

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