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846 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. LITTLE J. P. HALLIDAY D. C. GLENN. University Department of Surgery, Sydney, Australia. SIR,-I was interested to read the paper by Mr. Little and his colleagues (Sept. 27, p. 659) advocating a vigorous approach to pseudomyxoma peritonei. I agree that this malignant condition often runs quite a prolonged course, and that the patient can be kept reasonably comfortable for some time. A patient under my care was originally operated on three years ago, and was found to have pseudomyxoma peritonei due to a fairly well differentiated pseudomucinous cyst- adenocarcinoma of the ovary. The bulk of the tumour was removed, together with a large amount of mucous material. No cytotoxic agent was instilled in the peritoneum on that occasion, 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. Several times the thiotepa was discontinued, and several times blood-transfusions were necessary. Two years after the first operation the tumour began to grow rapidly and on Aug. 2, 1968, I carried out a further laparotomy, again removing about four litres of viscid material which was contained in a large thick-walled cystic cavity, itself densely adherent to the small and large intestines. It seemed to me that it would be impossible to remove the cyst safely and rather than subject the patient to repeated operations, I marsupialised the cyst in the left iliac fossa, producing a mucous 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 hospital on two occasions for blood-transfusions. There is now very little abnormal to feel within the abdomen and the mucous fistula seems not to trouble her. She wears only a small dressing under her corset which has to be changed once daily. Obviously there is a risk in prolonged cytotoxic drug therapy. On occasions this patient’s platelet-count has fallen below 20,000 and she has developed purpura, but this state seems to be easily reversible when the drug is 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 malignancy to warrant the risk. SIR,-Mr. Little and his colleagues suggest that most cases of pseudomyxoma peritonei are due to malignant tumours. I have recently examined the appendix in five cases of pesudomyxoma peritonei of appendiceal origin, and, in my opinion, none of these was due to a malignant tumour. I have also examined fifteen cases of adenocarci- noma of the appendix; in none of these was there any evidence of pseudomyxoma peritonei, and I have been unable 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 mucoceles and cystadenomas. Death is almost invariably due to intestinal obstruction, distant metastases are virtually unknown, long-term survival and relatively good physical well-being are common, and the scanty evidence that this condition is due to a malignant neoplasm is based almost entirely on histological interpretation. Semantics bedevil this condition, but I should like to make the following points: (1) the material in the abdominal cavity is mucin not pseudomucin; (2) pseudomyxoma peritonei no longer seems an appropriate term (mucinous or gelatinous ascites might perhaps be preferable); and (3) fig. 5 in the article appears to be a cystadenoma and not an adenocarcinoma of the appendix, and fig. 7 has the histological appearance of pseudomyxoma peritonei rather than 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 interesting comments. We agree with his suggestion that the term pseudomyxoma peritonei might well be dropped and replaced by one of the terms he suggests, particularly if, as he states, the gelatinous material is mucin and not pseudo- mucin. We would, however, disagree with some other points. Two instances of metastasising appendiceal neoplasms associated with the pseudomyxoma syndrome have been documented by Bernhardt and Young, and were cited 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 called carcinoma 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 original sections. Dr. Melcher rightly points out that the patho- logical interpretation he chooses is a matter of opinion, and it is important to stress that other pathologists may call the causative tumour malignant. The clinical implications of the 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 be defined in clinical terms. Finally, we agree completely that a central registry of cases would provide interesting and valuable information. HYDROXYUREA IN THE TREATMENT OF REFRACTORY PSORIASIS SIR,-The purpose of this communication is to report the efficacy of hydroxyurea in the treatment of refractory psoriasis. Hydroxyurea is a selective inhibitor of D.N.A. synthesis and has been shown to be an effective agent for the control of chronic granulocytic leukoemia.3 This agent was used to treat two patients with severe psoriasis. Case 1-A 55-year-old man presented at the University of Minnesota Hospital in June, 1965, with severe psoriasis of several years’ duration involving almost his entire body. This had been refractory to conventional therapy. Therapy was begun with hydroxyurea, 40 mg. per kg. for 6 days, and subsequently 20 mg. per kg. No change in haemoglobin or platelet-count was seen. The white-cell count fell initially from 9100 per c.mm. to 2350 per 1. 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

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846

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