1
25 1931, 1934, Weber 1943, Rosenbaum et al. 1953). However, carcinoid tumours are often associated with - carcinoma in other parts of the body (Pearson and Fitzgerald 1949, McKusick 1956), and a carcinoid may have been overlooked in some of the patients with symptoms suggestive of its presence. In our case a careful .search was made at necropsy, but no carcinoid tumour was found. Estimation of 5-H.i.A.A. (a metabolite of 5-hydroxy- tryptamine) in the urine is now generally regarded as - the most useful test for the presence of a carcinoid tumour and is thought to be specific. Two cases of carcinoma, however, have been reported (one of the breast and one of the larynx) (Clerc-Bory et al. 1954) in which urinary -chromatograms showed abnormally large amounts of 5-H.I.A.A. It was not stated whether the livers were involved by tumour or not. In a small series of cases of gastro- intestinal carcinoma with liver metastases (Snow et al. 1955), a slightly raised excretion of 5-H.I.A.A. was reported, but the levels were much lower than those found in cases of malignant carcinoid by the same authors. In another series of cases of carcinoma with metastases in the liver the urinary excretion of 5-H.i.A.A. was normal (Haverback et al. 1956). In our patient, a slight excess of 5-H.i.A.A. was found in the urine. The liver was extensively infiltrated by metastases from a bronchial carcinoma, but no carcinoid tumour was discovered. We conclude therefore that increased urinary excretion of 5-H.i.A.A. may occur with malignant tumours other than carcinoid. Summary The case is reported of a woman of 55 with a carcinoma of the bronchus. She presented the clinical features of transient Cushing’s syndrome and .had some symptoms suggestive of a carcinoid tumour. There was a slight excess of 5-H.i.A.A. in the urine, but a carcinoid tumour was not found at necropsy. We are grateful to Prof. J. H. Biggart for his advice on the pathological findings, to Prof. D. C. Harrison for the estima- tion of 5-H.I.A.A., to Mr. H. M. Stevenson for the broncho- scopy, and to Mr. R. G. Wood for the photograph. ADDENDUM Since this article was submitted; Boyland et al. (1956) have reported the detection of small amounts of 5- H.LA.A. in the urine of patients with carcinoma of bronchus and other sites, following the ingcstion of 2 g. of Mryptophan. In these patients significant amounts of 5-H.I.A.A. were not apparently found unless trypt- ophan had been ingested, although this is not definitely stated in every case. REFERENCES Arnett, J. H., Long, C. F. (1931) Amer. J. med. Sci. 182, 212. Bongiovanni, A. M., Eisenmenger, W. J. (1951) J. clin. Endocrin. 11, 152. Boyland, E., Gasson, J. E., Williams, D. C. (1956) Lancet, ii, 975. Brown, W. H. (1928) Ibid, ii, 1022. Cassidy, M. A. (1931) Proc. R. Soc. Med. 24, 139, 920. — (1934) Ibid, 27, 220. Clerc-Bory, M., Pachéco, H., Mentzer, C. (1954) C.R. Acad. Sci., Paris, 238, 525. Crispell, K. R., Parson, W. (1952) Amer. J. Med. 13, 247. Haverback, B. J., Sjoerdsma, A., Terry, L. L. (1956) New Engl. J. Med. 255, 270. Heinbecker, P. (1944) Medicine, 23, 225. Jepson, R. P. (1956) Personal communication. McKusick, V. A. (1956) Bull. Johns Hopk. Hosp. 98, 13. Pearson, C. M., Fitzgerald, P. J. (1949) Cancer, 2, 1005. Prunty, F. T. G. (1956) Brit. med. J. ii, 615, 673. Rosenbaum, F. F., Santer, D. G., Claudon, D. B. (1953) J. Lab. clin. Med. 42, 941. Snow, P. J. D., Lennard-Jones, J. E., Curzon, G., Stacey, R. S. (1955) Lancet, ii, 1004. Thorne, M. G. (1952) Guy’s Hosp. Rep., 101, 251. Weber, F. P. (1943) Med. Pr. 210, 219. Zondek, H. (1923) Die Krankheiten der endocrinen Drüsen, Berlin, p. 371. — Leszynsky, H. E. (1956) Brit. med. J. i, 197. UTERINE MYOMA AS A HEREDITARY DISEASE L. E. WEAR M.B. Leeds, D.C.H. GENERAL PRACTITIONER, CREWKERNE, SOMERSET UTERINE fibromatosis is not usually considered to be hereditary, but the occurrence of uterine fibroids in more than one member of a family has occasionally been noticed. Lockyer (1918) stated : " It is certainly striking how fibroids run in families. A patient of my own is one of five sisters, four of whom have had uterine myoma, and the fifth refuses investigation but is thought to be similarly afflicted." Lagrona-Weill-Halle (1950) described two sisters, aged 19 and 22, with identical clinical features due to multiple small intramural uterine fibroids. No family history was given. All the affected members of the family described here that I have managed to talk to had large uterine fibroids which produced a palpable abdominal tumour but no abnormality of the menstrual cycle. They remained undiscovered until either they complicated a pregnancy or the abdomen was examined for some other reason. The Family Generation iv (see figure) are all aged less than 15 and are excluded from the present survey. At least 9 of the 15 females in generations I-III have had uterine fibroids. I have talked to 5 adult members of the family independently, and all of these named the same relations as having had uterine fibroids ; I have personally attended 3 of these patients, and 2 other cases were confirmed by the patient’s own doctor. A typical case is that of III.3: During a routine examination while she was in bed with influenza in March, 1956, a firm abdominal swelling extending up to the umbilicus was palpated. That this was caused by several uterine fibroids was confirmed on bimanual examina- tion. The menses were regular and normal in amount. At operation in August, 1956, one large intramural fibroid and two medium-sized subserous fibroids were found, and subtotal hysterectomy was done. Discussion 1.1 in the first generation had 7 children before under- going a hysterectomy at the age of 44. This is an unusual number for a diseased uterus to bear. Usually there are no, or only 1 or 2, children of a mother with uterine fibroids, as in generation 11. This fact makes the disease self-limiting by reducing the number of progeny. Uterine fibroids are rarely discovered before the fourth or fifth decade of life and even then may go unnoticed and symptomless. It is therefore difficult to piece together a full hereditary picture ; some with fibroids may be missed, particularly if they are unmarried or childless. From the family tree it looks as if the condition may be transmitted through males as well as females : IL4, a

Addendum

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25

1931, 1934, Weber 1943, Rosenbaum et al. 1953).However, carcinoid tumours are often associated with- carcinoma in other parts of the body (Pearson andFitzgerald 1949, McKusick 1956), and a carcinoid mayhave been overlooked in some of the patients withsymptoms suggestive of its presence. In our case a careful.search was made at necropsy, but no carcinoid tumourwas found.

Estimation of 5-H.i.A.A. (a metabolite of 5-hydroxy-tryptamine) in the urine is now generally regarded as- the most useful test for the presence of a carcinoid tumourand is thought to be specific. Two cases

of carcinoma, however, have been reported(one of the breast and one of the larynx)(Clerc-Bory et al. 1954) in which urinary-chromatograms showed abnormally largeamounts of 5-H.I.A.A. It was not statedwhether the livers were involved by tumouror not. In a small series of cases of gastro-intestinal carcinoma with liver metastases

(Snow et al. 1955), a slightly raised excretionof 5-H.I.A.A. was reported, but the levelswere much lower than those found in cases of

malignant carcinoid by the same authors.In another series of cases of carcinoma withmetastases in the liver the urinary excretionof 5-H.i.A.A. was normal (Haverback et al.1956).In our patient, a slight excess of 5-H.i.A.A. was found

in the urine. The liver was extensively infiltrated bymetastases from a bronchial carcinoma, but no carcinoidtumour was discovered. We conclude therefore thatincreased urinary excretion of 5-H.i.A.A. may occur withmalignant tumours other than carcinoid.

SummaryThe case is reported of a woman of 55 with a carcinoma

of the bronchus. She presented the clinical features oftransient Cushing’s syndrome and .had some symptomssuggestive of a carcinoid tumour. There was a slightexcess of 5-H.i.A.A. in the urine, but a carcinoid tumourwas not found at necropsy.We are grateful to Prof. J. H. Biggart for his advice on the

pathological findings, to Prof. D. C. Harrison for the estima-tion of 5-H.I.A.A., to Mr. H. M. Stevenson for the broncho-scopy, and to Mr. R. G. Wood for the photograph.

ADDENDUM

Since this article was submitted; Boyland et al. (1956)have reported the detection of small amounts of 5-H.LA.A. in the urine of patients with carcinoma ofbronchus and other sites, following the ingcstion of 2 g.of Mryptophan. In these patients significant amountsof 5-H.I.A.A. were not apparently found unless trypt-ophan had been ingested, although this is not definitelystated in every case.

REFERENCES

Arnett, J. H., Long, C. F. (1931) Amer. J. med. Sci. 182, 212.Bongiovanni, A. M., Eisenmenger, W. J. (1951) J. clin. Endocrin.

11, 152.Boyland, E., Gasson, J. E., Williams, D. C. (1956) Lancet, ii, 975.Brown, W. H. (1928) Ibid, ii, 1022.Cassidy, M. A. (1931) Proc. R. Soc. Med. 24, 139, 920.

— (1934) Ibid, 27, 220.Clerc-Bory, M., Pachéco, H., Mentzer, C. (1954) C.R. Acad. Sci.,

Paris, 238, 525.Crispell, K. R., Parson, W. (1952) Amer. J. Med. 13, 247.Haverback, B. J., Sjoerdsma, A., Terry, L. L. (1956) New Engl.

J. Med. 255, 270.Heinbecker, P. (1944) Medicine, 23, 225.Jepson, R. P. (1956) Personal communication.McKusick, V. A. (1956) Bull. Johns Hopk. Hosp. 98, 13.Pearson, C. M., Fitzgerald, P. J. (1949) Cancer, 2, 1005.Prunty, F. T. G. (1956) Brit. med. J. ii, 615, 673.Rosenbaum, F. F., Santer, D. G., Claudon, D. B. (1953) J. Lab.

clin. Med. 42, 941.Snow, P. J. D., Lennard-Jones, J. E., Curzon, G., Stacey, R. S.

(1955) Lancet, ii, 1004.Thorne, M. G. (1952) Guy’s Hosp. Rep., 101, 251.Weber, F. P. (1943) Med. Pr. 210, 219.Zondek, H. (1923) Die Krankheiten der endocrinen Drüsen, Berlin,

p. 371.— Leszynsky, H. E. (1956) Brit. med. J. i, 197.

UTERINE MYOMA AS A HEREDITARY

DISEASE

L. E. WEARM.B. Leeds, D.C.H.

GENERAL PRACTITIONER, CREWKERNE, SOMERSET

UTERINE fibromatosis is not usually considered to behereditary, but the occurrence of uterine fibroids in morethan one member of a family has occasionally beennoticed.

Lockyer (1918) stated : " It is certainly striking how fibroidsrun in families. A patient of my own is one of five sisters,four of whom have had uterine myoma, and the fifth refuses

investigation but is thought to be similarly afflicted."Lagrona-Weill-Halle (1950) described two sisters, aged 19

and 22, with identical clinical features due to multiple smallintramural uterine fibroids. No family history was given.

All the affected members of the family described herethat I have managed to talk to had large uterine fibroidswhich produced a palpable abdominal tumour but noabnormality of the menstrual cycle. They remainedundiscovered until either they complicated a pregnancyor the abdomen was examined for some other reason.

The FamilyGeneration iv (see figure) are all aged less than 15

and are excluded from the present survey. At least 9of the 15 females in generations I-III have had uterinefibroids. I have talked to 5 adult members of the familyindependently, and all of these named the same relationsas having had uterine fibroids ; I have personally attended3 of these patients, and 2 other cases were confirmed bythe patient’s own doctor.A typical case is that of III.3:

During a routine examination while she was in bed withinfluenza in March, 1956, a firm abdominal swelling extendingup to the umbilicus was palpated. That this was caused byseveral uterine fibroids was confirmed on bimanual examina-tion. The menses were regular and normal in amount. At

operation in August, 1956, one large intramural fibroid andtwo medium-sized subserous fibroids were found, and subtotalhysterectomy was done.

Discussion1.1 in the first generation had 7 children before under-

going a hysterectomy at the age of 44. This is an unusualnumber for a diseased uterus to bear. Usually there areno, or only 1 or 2, children of a mother with uterinefibroids, as in generation 11. This fact makes the disease

self-limiting by reducing the number of progeny.Uterine fibroids are rarely discovered before the fourth

or fifth decade of life and even then may go unnoticedand symptomless. It is therefore difficult to piecetogether a full hereditary picture ; some with fibroidsmay be missed, particularly if they are unmarried orchildless.From the family tree it looks as if the condition may

be transmitted through males as well as females : IL4, a