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Unfortunately it is usually impossible to judge from a chair-man’s remarks whether one’s paper has been good or bad-and too many friends are not critical enough.
D. DAVIES.Ransom Hospital,
Rainworth, Nottinghamshire.
PSEUDOMYXOMA PERITONEI
J. MAGELL.Royal Infirmary,
Blackburn.
D. H. MELCHER.
Department of Pathology,Royal Sussex County Hospital,
Brighton.
J. M. LITTLEJ. P. HALLIDAYD. C. GLENN.
University Department of Surgery,Sydney, Australia.
SIR,-I was interested to read the paper by Mr. Littleand his colleagues (Sept. 27, p. 659) advocating a vigorousapproach to pseudomyxoma peritonei. I agree that this
malignant condition often runs quite a prolonged course,and that the patient can be kept reasonably comfortablefor some time.A patient under my care was originally operated on three
years ago, and was found to have pseudomyxoma peritoneidue to a fairly well differentiated pseudomucinous cyst-adenocarcinoma of the ovary. The bulk of the tumour wasremoved, together with a large amount of mucous material.No cytotoxic agent was instilled in the peritoneum on thatoccasion, but subsequently she received thiotepa, 15 mg.intramuscularly twice weekly, provided that her white-cell count was over 3000 per c.mm. and that her haemo-
globin and platelet-count were satisfactory. Severaltimes the thiotepa was discontinued, and several timesblood-transfusions were necessary. Two years after thefirst operation the tumour began to grow rapidly and onAug. 2, 1968, I carried out a further laparotomy, againremoving about four litres of viscid material which wascontained in a large thick-walled cystic cavity, itself denselyadherent to the small and large intestines. It seemed tome that it would be impossible to remove the cyst safelyand rather than subject the patient to repeated operations,I marsupialised the cyst in the left iliac fossa, producing amucous fistula. The cyst was irrigated with thiotepa.The patient has remained on thiotepa since that time,
and in the past twelve months she has been in hospitalon two occasions for blood-transfusions. There is nowvery little abnormal to feel within the abdomen and themucous fistula seems not to trouble her. She wears onlya small dressing under her corset which has to be changedonce daily. Obviously there is a risk in prolonged cytotoxicdrug therapy. On occasions this patient’s platelet-counthas fallen below 20,000 and she has developed purpura,but this state seems to be easily reversible when the drugis stopped and transfusions of fresh blood are given.The possibility of a tumour fungating through a fistula
is an argument against this line of treatment, but pseudo-myxoma appears to be of sufficiently low-grade malignancyto warrant the risk.
SIR,-Mr. Little and his colleagues suggest that mostcases of pseudomyxoma peritonei are due to malignanttumours. I have recently examined the appendix in fivecases of pesudomyxoma peritonei of appendiceal origin,and, in my opinion, none of these was due to a malignanttumour. I have also examined fifteen cases of adenocarci-noma of the appendix; in none of these was there anyevidence of pseudomyxoma peritonei, and I have beenunable to discover any published account of a meta-
stasising appendiceal neoplasm associated with pseudo-myxoma peritonei.The tumours of the appendix which most commonly
produce pseudomyxoma peritonei are benign mucocelesand cystadenomas. Death is almost invariably due to
intestinal obstruction, distant metastases are virtuallyunknown, long-term survival and relatively good physical
well-being are common, and the scanty evidence that thiscondition is due to a malignant neoplasm is based almostentirely on histological interpretation.
Semantics bedevil this condition, but I should like tomake the following points: (1) the material in the abdominalcavity is mucin not pseudomucin; (2) pseudomyxomaperitonei no longer seems an appropriate term (mucinousor gelatinous ascites might perhaps be preferable); and(3) fig. 5 in the article appears to be a cystadenoma and notan adenocarcinoma of the appendix, and fig. 7 has the
histological appearance of pseudomyxoma peritonei ratherthan of adenocarcinoma.There would be much to be said for having a central
registry of cases of this unusual condition.
** This letter was shown to Mr. Little and his colleagues,whose reply follows.-ED. L.
SIR,-We are grateful to Dr. Melcher for his interestingcomments. We agree with his suggestion that the termpseudomyxoma peritonei might well be dropped andreplaced by one of the terms he suggests, particularly if, ashe states, the gelatinous material is mucin and not pseudo-mucin. We would, however, disagree with some otherpoints. Two instances of metastasising appendicealneoplasms associated with the pseudomyxoma syndromehave been documented by Bernhardt and Young, and werecited in our article. Dr. Melcher has interpreted the histo-logy of figs. 5 and 7 in a different way from our own
pathologists, who feel that both specimens must be calledcarcinoma because invasion can be demonstrated in both.This may not be obvious in the black-and-white reproduc-tions published, but can be clearly seen in the originalsections. Dr. Melcher rightly points out that the patho-logical interpretation he chooses is a matter of opinion, andit is important to stress that other pathologists may call thecausative tumour malignant. The clinical implications ofthe term are a little different in this condition. Because
pathologists cannot always agree on the diagnosis of
malignancy, we stress that the condition may best bedefined in clinical terms. Finally, we agree completely thata central registry of cases would provide interesting andvaluable information.
HYDROXYUREA IN THE TREATMENT OF
REFRACTORY PSORIASIS
SIR,-The purpose of this communication is to report theefficacy of hydroxyurea in the treatment of refractorypsoriasis. Hydroxyurea is a selective inhibitor of D.N.A.synthesis and has been shown to be an effective agent forthe control of chronic granulocytic leukoemia.3 This agentwas used to treat two patients with severe psoriasis.
Case 1-A 55-year-old man presented at the University ofMinnesota Hospital in June, 1965, with severe psoriasis of severalyears’ duration involving almost his entire body. This had beenrefractory to conventional therapy. Therapy was begun withhydroxyurea, 40 mg. per kg. for 6 days, and subsequently 20 mg.per kg. No change in haemoglobin or platelet-count was seen. Thewhite-cell count fell initially from 9100 per c.mm. to 2350 per1. Bernhardt, H., Young, J. H. Am. J. Surg. 1965, 109, 235.2. Yarbro, J. W., Kennedy, B. J., Barnum, C. P. Proc. natn. Acad. Sci.
U.S.A. 1965, 53, 1033.3. Kennedy, B. J., Yarbro, J. W. J. Am. med. Ass. 1966, 195, 1038