Squamous cell carcinoma arising in a meningomyolocele JAN M. SAKSUN, MD; BENJAMIN K. FISHER, MD, FRCP[C]
Squamous cell carcinoma developed in the meningomyelocele of a 25-year-old man. This is the third such case reported. The possibility of malignant disease arising in this congenital defect must be taken into account when treatment is being considered. Un epitheliome spinocellulaire est apparu dans le meningomyelocele d'un homme de 25 ans. C'est le troisieme cas rapporte. La possibilite d'une degenerescence maligne d6veloppant dans cette anomalie cong6nitale dolt .tre price en consid6ration quand le traitement est envisage. Squamous cell carcinoma is a possible serious complication of chronic scars, ulcers and areas of inflammation. We present a case in which malignant change arose in a congenital defect, a meningomyelocele, after years of mechanical irritation and chronic bacterial infection.
Case report A 25-year-old man was seen in consultation during an admission for hemodialysis because of a tumour in the lumbosacral area. He was the fourth child of nonconsanguineous parents. At the time of his birth he was noted to have a lumbosacral meningomyelocele, but no medical or surgical treatment was instituted in view of the severity of the defects. At the age of 9 years he was seen by medical specialists because of various problems. They found him to be a bright child and agile with his braces and crutches. The lumbosacral area was deformed by a large meningomyelocele 6 cm in diameter, and a decubitus ulcer was present distal to the lesion. Flaccid paralysis of both legs, fixed pelvic obliquity, unstable hip joints, and deformed feet and ankles were noted. Rectal examination revealed little inherent tone of the anal sphincter. Neurologic consultation confirmed the presence of a meningomyelocele with cauda equina involvement. From the division of dermatology, department of medicine, Wellesley Hospital, Toronto, and the faculty of medicine, University of Toronto Reprint requests to: Dr. Benjamin K. Fisher, Jones Building, Ste. 326, The Wellesley Hospital, 160 Wellesley St. E, Toronto, Ont. M4Y 1J3
There were no signs of hydrocephalus. The consultants thought that surgical treatment of the meningomyelocele was contraindicated because of the significant risk of altering the well compensated dynamics of the cerebrospinal fluid. The boy was followed up by his family physician until the age of 17 years, when, because of recurrent urinary tract infections, he was hospitalized for evaluation of renal function. Renal insufficiency was found; the blood urea nitrogen (BUN) concentration was 49 mg/dL (normal, 8 to 21 mg/dL) and the serum creatinine concentration 2.9 mg/dL (normal, 0.9 to 1.7 mg/dL). Roentgenograms showed minimal renal function bilaterally and gross hydronephrosis. Therefore bilateral cutaneous ureterostomies were constructed to permit free drainage of urine. The orthopedic consultants considered operative procedures to be indicated since the boy was no longer able to walk with crutches. They were reluctant to proceed, however, until the meningomyelocele was repaired because it was infected and thus, they argued, presented a considerable risk of intraoperative infection. The neurosurgeon declined to undertake the repair, noting that the infection was superficial and not associated with meningitis. Surgical interference, he thought, could allow bacteria to enter the spinal canal, the result being meningitis or derangement of the well compensated flow of cerebrospinal fluid, with the development of hydrocephalus and mental deterioration. Three years later the young man was admitted to hospital because of renal failure. Peritoneal dialysis was begun and later was replaced by hemodialysis. At the time of admission a purulent discharge from the meningomyelocele was noted; compresses were applied locally and antibiotics were given by mouth. The dermatology division was consulted 5 years later, during one of the admissions for hemodialysis. The patient was oriented and alert, but had a pale, sallow complexion. Three infected decubitus ulcers were noted in addition to scoliosis to the left, severe kyphosis of the spine, and extreme wasting of the musculature below the waist and of the legs. In the lumbosacral area was a fungating tumour 10 cm in diameter, consisting of friable red tissue with heaped-up edges, on a slightly larger, moist, ulcerated base
(Fig. 1). The area smelled foul and was constantly draining serosanguineous fluid. The clinical diagnosis was squamous cell carcinoma. Further physical, laboratory and radiologic studies showed no evidence of metastases. Two biopsies were performed. Histologic examination of sections from the large wedge-shaped specimens showed hyperkeratosis alternating with parakeratosis over a slightly acanthotic epidermis. From the inferior border of the epidermis thin, finger-like projections of epidermal cells extended irregularly deep into the dermis and subcutaneous tissue, forming a lacelike pattern. These cords of tumour contained degenerating epidermal cells and "Horn pearls". A few atypical cells and mitoses were seen. The appearance was diagnostic of squamous cell carcinoma (Fig. 2). Both the plastic surgeons and the neurosurgeons thought that it would be technically impossible to remove the tumour, so radiotherapy, 800 rads every 2 weeks for a total of 2400 rads, was given. Less than 2 weeks after the radiotherapy was completed the patient was discharged from hospital, but within 4 weeks he began to complain of drowsiness and headache. He suffered a convulsion and was treated by his family physician with diphenylhydantom and antibiotics given systemically.
FIG. 1-Initial appearance of tumour in lumbosacral area, with friable, verrucous surface and suppuration.
CMA JOURNAL/OCTOBER 7, 1978/VOL. 119 739
He was readmitted to hospital semico- is called "spina bifida cystica". Some matose almost 8 weeks after discharge. 75% of lesions so designated are A sample of cerebrospinal fluid ob- meningomyeloceles (defects of skin, tained by lumbar puncture was found lack of fusion of vertebral arches, to be normal by means of India ink cystic dilatation of the meninges with preparation, routine bacterial smear, bacterial and fungal cultures and ex- myelodysplasia and neurologic signs) amination for malignant cells. The glu- and 25% are meningoceles (lack of cose concentration was 53 mg/ dL (nor- fusion of vertebral arches and cystic mal, 40 to 75 mg/dL), the protein dilatation of the meninges but no concentration 52 mg/dL (normal, 15 neurologic signs since the meningeal to 45 mg/dL) and the cell count in- sac does not contain part of the cord). creased, at 130 leukocytes/mm', with Together the cystic and occult types 85% polymorphonuclear forms and of spina bifida make up 50% of the 15% monocytes (normal, 0 to 4 cells! malformations of the central nervous mm3). At this time the tumour was system; central nervous system malnoted to be somewhat small and un- formations account for 15% of all dergoing necrosis (Fig. 3). The patient died 4 days later of developmental malformations.5 Our patient had meningomyelocele meningitis. Permission was not obin which there was lack of fusion of tained for postmortem examination. vertebral arches, myelodysplasia, cystic dilatation of the meninges with Discussion neural elements in the cyst roof, and The terminology of defects of the a defect in the overlying skin. spinal canal has been reviewed reThe clinical picture and the progcently by several authors.14 The gen- nosis of meningomyelocele (which eral term used for abnormalities in always includes some degree of neuwhich there is a failure of midline rologic impairment) depend on the fusion of the spinal arches is "spina level of the lesion and the degree of bifida". When the meninges and myelodysplasia. spinal cord are involved the lesion Several studies have shown that
once a child has been born with a neural tube defect the risk of recurrence in the same family is 4% to 5%, or 7 to 40 times that normally expected in matched families having no such child."3'6 Thus, genetic counselling is essential to a family in which an affected child has been born. Recently prenatal detection has become possible with amniocentesis, which allows measurement of the abnormally increased concentration of a-fetoprotein in the amniotic fluid.7-'0 A search of the literature revealed
only two cases similar to the one we have described. In the case reported by Thorp11 surgical treatment was chosen initially for a 26-year-old man with a carcinoma at the site of a lumbar meningomyelocele. Six months after resection the tumour recurred and was treated with radiotherapy. Later the tumour appeared again, necessitating further surgery. Three weeks postoperatively the patient died of septicemia. In the case described by Pope and Todorov1' a squamous cell carcinoma developed at the site of a cervical meningomyelocele in a 37-year-old man. This lesion was easily excised, without complications, because the defect was a meningocele and hence did not contain neural elements or connect with the spinal canal. Two cases of malignant teratomas
occurring in meningoceles and result-
FIG. 3-Tumour smaller and undergoing necrosis 8 weeks after completion of radiotherapy.
South Med J 58: 779, 1965 ing in death have also been re- 10. FERNHOFF PM, OAKLEY GP: Alphafetoprotein as predictor of fetal 19. LIDDELL K: Malignant change in ported.13'14 neural-tube defects. Lancet 2: 368, chronic varicose ulceration. PractiOne must be aware of possible 1975 tioner 215: 335, 1975 neoplastic change in untreated me- 11. THORP RH: Carcinoma associated 20. HALLIDAY JP: Squamous cell carciningomyeloceles after years of mewith myelomeningocele. Case report. noma in a venous ulcer. Med J A ust chanical irritation and chronic bacJ Neurosurg 27: 446, 1967 1: 449, 1968 terial infection. The defect, lacking 12. POPE M, ToDoRov AB: Cutaneous 21. BROWN HW, RIVERA J: Epidermoid a protective epithelial cover, must be squamous cell carcinoma as a rare carcinoma arising in a pilonidal sinus. complication of cervical meningocele. viewed regularly with a high degree Report of a case and review of the Birth Dejects 11: 336, 1975 of suspicion, as with other chronic literature. mt Surg 50: 435, 1968 ulcers; squamous cell carcinoma is a 13. LOVE JG: Delayed malignant devel- 22. DONSKY HJ, MENDELSON CG: Squamopment of a congenital teratoma with ous cell carcinoma as a complication well known complication of burn and spina bifida. Case report. J Neurosurg hidradenitis suppurativa. Arch of other .117 chronic venous ul29: 532, 1968 Dermatol 90: 488, 1964 cers,18'20 pilonidal sinuses,21 long- 14. MICKLE JP, MCLENNAN JE: Malignant 23. HUMPHREY PLAYFORm U, H, standing bacterial and fungal infecteratoma arising within a lipomeninLEAvELL UW Squamous JR: cell carcitions,'2.' vaccination scars26'27 and gocele. Case report. J Neurosurg 43: noma arising in hidradenitis suppura761, 1975 even tattoos.28 Once change is noted, tivum. Arch Dermatol 100: 59, 1969 biopsies should be done to establish 15. ARONS MS, LYNCH JB, LEWIS SR, et 24. FORSTROM L: Carcinomatous changes al: Scar tissue carcinoma: Part I. A a histologic diagnosis. If malignancy in lupus vulgaris. Ann Clin Res 1: clinical study with special reference is established, an intensive search for 213, 1969 to burn scar carcinoma. Ann Surg metastases, lymph node involvement 25. CAPLAN RM: Epidermoid carcinoma 161: 170, 1965 arising in extensive chromoblastomyand local invasion must be made for 16. BOSTwICK J iii, PENDERGRAST WJ, cosis. Arch Dermatol 97: 38, 1968 proper staging and subsequent treatVASCONEZ LO: Marjolin's ulcer: an MARMELZAT WL: Malignant tumors 26. ment. immunologically privileged tumor? in smallpox vaccination scars. Ibid, p Plast Reconstr Surg 57: 66, 1976 We thank Dr. Clair Williams for his 17. DIDOLKAR MS, GERNER RE, MOORE 400 permission to publish this case. 27. REED WB, WILsON-JONEs E: MaligGE: Epidermolysis bullosa dystronant tumors as late complication of phica and epithelioma of the skin: References vaccination. Arch Dermatol 98: 132, review of published cases and report 1. American Academy of Orthopedic Surgeons: Symposium on Myelomeningocele, Mosby, St. Louis, 1972, pp 1-20 2. FREEMAN JM: Practical Management of Meningomyelocele, Univ Park Pr,
of an additional patient. Cancer 33: 198, 1974 18. PENNELL TC, HIGHTOWER F: Malignant changes in post-phlebitic ulcers.
1968 28. MCQUARRIE DG: Squamous-cell carcinoma arising in a tattoo. Minn Med 49: 799, 1966
Baltimore, Md, 1974, pp 1-22
3. SMITh ED (ed): Spida Bitida and the Total Care of Spinal Myelomeningocele,
CC Thomas, Springfield, Ill,
Historical notes typhoid fever
1965, pp 3-46 4. HARRIS HW, MILLER OF: Midline
cutaneous and spinal defects. Arch Dermatol 112: 1724, 1976 5. HEMMER R: Meningoceles and myeloceles, in Progress in Neurological Surgery, vol 4, KRAYENBUHL H (ed), Phiebig, White Plains, NY, 1971, pp
192-226 6. SPECHT EE, GOODNER EK, TANAGHO EA, et al: Myelomeningocele: a symposium on orthopedic, ophthalmologic, urologic, psychological and social, neurosurgical and general considerations. West J Med 121: 281, 1974 7. BROCK DJH, SUTCLIFFE RG: Alphafetoprotein in the antenatal diagnosis of anencephaly and spina bifida. Lancet 2: 197, 1972 8. COLTART TM, SELLER MJ, SINGER
JD, et al: Amniotic fluid concentrations of alpha-fetoprotein (AEP) in early normal pregnancy, and pregnancy complicated by neural tube defects. A review of 18 months experience. Guy's Hasp Rep 123: 121, 1974 9. MILUNSKY A, ALPERT E: The value
of alpha-fetoprotein in the prenatal diagnosis of neural tube defects. J Pediatr 84: 889, 1974
In 1856 William Bud, a British small-town practitioner, published a series of articles in which he demonstrated that in the stools of patients with typhoid there was an agent that carried the disease to other patients (Dowling HF: Fighting Infection, Harvard University Press, Cambridge, Massachusetts, 1977). He evolved a system for preventing the spread of typhoid that included boiling contaminated linen, chemically disinfecting the discharges from typhoid patients and having attendants wash their hands. He considered that "the sewer may be looked upon... as a direct continuation of the diseased intestine" and urged that water and milk be boiled during an epidemic. Charles Murchison, a person of some prominence, dismissed these views and held that putrefaction was the cause of typhoid; Murchison's views prevailed for some time. In the early 1900s and until recently the greatest health problem
related to typhoid was deciding on the proper management of carriers of the disease. In 1906 George Soper of New York City's health departm.nt investigated six cases of typhoid that had occurred in one household. He learned that a recently employed Irish cook had left abruptly after a stay of 3 weeks. When he finally traced the cook he discovered that she had been the cause of seven epidemics of typhoid in 6 years. Her name was Mary Mallon, but she came to be known as Typhoid Mary. In all, she was responsible for approximately 60 cases of typhoid, 3 of which were fatal, in New York City. Once discovered, Mary was kept in hospital for 3 years, but she discharged herself and, against medical advice, took a job involving the handling of food; this led to a further series of epidemics. Cholecystectomy was the only known treatment for carriers at the time, but Mary would have no part of it; she spent the last 23 years of her life in hospital under what we today would call protective custody.u
CMA JOURNAL/OCTOBER 7, 1978/VOL. 119 741
Squamous cell carcinoma developed in the meningomyelocele of a 25-year-old man. This is the third such case reported. The possibility of malignant disease arising in this congenital defect must be taken into account when treatment is being considered. Un epitheliome spinocellulaire est apparu dans le meningomyelocele d'un homme de 25 ans. C'est le troisieme cas rapporte. La possibilite d'une degenerescence maligne d6veloppant dans cette anomalie cong6nitale dolt .tre price en consid6ration quand le traitement est envisage. Squamous cell carcinoma is a possible serious complication of chronic scars, ulcers and areas of inflammation. We present a case in which malignant change arose in a congenital defect, a meningomyelocele, after years of mechanical irritation and chronic bacterial infection.
Case report A 25-year-old man was seen in consultation during an admission for hemodialysis because of a tumour in the lumbosacral area. He was the fourth child of nonconsanguineous parents. At the time of his birth he was noted to have a lumbosacral meningomyelocele, but no medical or surgical treatment was instituted in view of the severity of the defects. At the age of 9 years he was seen by medical specialists because of various problems. They found him to be a bright child and agile with his braces and crutches. The lumbosacral area was deformed by a large meningomyelocele 6 cm in diameter, and a decubitus ulcer was present distal to the lesion. Flaccid paralysis of both legs, fixed pelvic obliquity, unstable hip joints, and deformed feet and ankles were noted. Rectal examination revealed little inherent tone of the anal sphincter. Neurologic consultation confirmed the presence of a meningomyelocele with cauda equina involvement. From the division of dermatology, department of medicine, Wellesley Hospital, Toronto, and the faculty of medicine, University of Toronto Reprint requests to: Dr. Benjamin K. Fisher, Jones Building, Ste. 326, The Wellesley Hospital, 160 Wellesley St. E, Toronto, Ont. M4Y 1J3
There were no signs of hydrocephalus. The consultants thought that surgical treatment of the meningomyelocele was contraindicated because of the significant risk of altering the well compensated dynamics of the cerebrospinal fluid. The boy was followed up by his family physician until the age of 17 years, when, because of recurrent urinary tract infections, he was hospitalized for evaluation of renal function. Renal insufficiency was found; the blood urea nitrogen (BUN) concentration was 49 mg/dL (normal, 8 to 21 mg/dL) and the serum creatinine concentration 2.9 mg/dL (normal, 0.9 to 1.7 mg/dL). Roentgenograms showed minimal renal function bilaterally and gross hydronephrosis. Therefore bilateral cutaneous ureterostomies were constructed to permit free drainage of urine. The orthopedic consultants considered operative procedures to be indicated since the boy was no longer able to walk with crutches. They were reluctant to proceed, however, until the meningomyelocele was repaired because it was infected and thus, they argued, presented a considerable risk of intraoperative infection. The neurosurgeon declined to undertake the repair, noting that the infection was superficial and not associated with meningitis. Surgical interference, he thought, could allow bacteria to enter the spinal canal, the result being meningitis or derangement of the well compensated flow of cerebrospinal fluid, with the development of hydrocephalus and mental deterioration. Three years later the young man was admitted to hospital because of renal failure. Peritoneal dialysis was begun and later was replaced by hemodialysis. At the time of admission a purulent discharge from the meningomyelocele was noted; compresses were applied locally and antibiotics were given by mouth. The dermatology division was consulted 5 years later, during one of the admissions for hemodialysis. The patient was oriented and alert, but had a pale, sallow complexion. Three infected decubitus ulcers were noted in addition to scoliosis to the left, severe kyphosis of the spine, and extreme wasting of the musculature below the waist and of the legs. In the lumbosacral area was a fungating tumour 10 cm in diameter, consisting of friable red tissue with heaped-up edges, on a slightly larger, moist, ulcerated base
(Fig. 1). The area smelled foul and was constantly draining serosanguineous fluid. The clinical diagnosis was squamous cell carcinoma. Further physical, laboratory and radiologic studies showed no evidence of metastases. Two biopsies were performed. Histologic examination of sections from the large wedge-shaped specimens showed hyperkeratosis alternating with parakeratosis over a slightly acanthotic epidermis. From the inferior border of the epidermis thin, finger-like projections of epidermal cells extended irregularly deep into the dermis and subcutaneous tissue, forming a lacelike pattern. These cords of tumour contained degenerating epidermal cells and "Horn pearls". A few atypical cells and mitoses were seen. The appearance was diagnostic of squamous cell carcinoma (Fig. 2). Both the plastic surgeons and the neurosurgeons thought that it would be technically impossible to remove the tumour, so radiotherapy, 800 rads every 2 weeks for a total of 2400 rads, was given. Less than 2 weeks after the radiotherapy was completed the patient was discharged from hospital, but within 4 weeks he began to complain of drowsiness and headache. He suffered a convulsion and was treated by his family physician with diphenylhydantom and antibiotics given systemically.
FIG. 1-Initial appearance of tumour in lumbosacral area, with friable, verrucous surface and suppuration.
CMA JOURNAL/OCTOBER 7, 1978/VOL. 119 739
He was readmitted to hospital semico- is called "spina bifida cystica". Some matose almost 8 weeks after discharge. 75% of lesions so designated are A sample of cerebrospinal fluid ob- meningomyeloceles (defects of skin, tained by lumbar puncture was found lack of fusion of vertebral arches, to be normal by means of India ink cystic dilatation of the meninges with preparation, routine bacterial smear, bacterial and fungal cultures and ex- myelodysplasia and neurologic signs) amination for malignant cells. The glu- and 25% are meningoceles (lack of cose concentration was 53 mg/ dL (nor- fusion of vertebral arches and cystic mal, 40 to 75 mg/dL), the protein dilatation of the meninges but no concentration 52 mg/dL (normal, 15 neurologic signs since the meningeal to 45 mg/dL) and the cell count in- sac does not contain part of the cord). creased, at 130 leukocytes/mm', with Together the cystic and occult types 85% polymorphonuclear forms and of spina bifida make up 50% of the 15% monocytes (normal, 0 to 4 cells! malformations of the central nervous mm3). At this time the tumour was system; central nervous system malnoted to be somewhat small and un- formations account for 15% of all dergoing necrosis (Fig. 3). The patient died 4 days later of developmental malformations.5 Our patient had meningomyelocele meningitis. Permission was not obin which there was lack of fusion of tained for postmortem examination. vertebral arches, myelodysplasia, cystic dilatation of the meninges with Discussion neural elements in the cyst roof, and The terminology of defects of the a defect in the overlying skin. spinal canal has been reviewed reThe clinical picture and the progcently by several authors.14 The gen- nosis of meningomyelocele (which eral term used for abnormalities in always includes some degree of neuwhich there is a failure of midline rologic impairment) depend on the fusion of the spinal arches is "spina level of the lesion and the degree of bifida". When the meninges and myelodysplasia. spinal cord are involved the lesion Several studies have shown that
once a child has been born with a neural tube defect the risk of recurrence in the same family is 4% to 5%, or 7 to 40 times that normally expected in matched families having no such child."3'6 Thus, genetic counselling is essential to a family in which an affected child has been born. Recently prenatal detection has become possible with amniocentesis, which allows measurement of the abnormally increased concentration of a-fetoprotein in the amniotic fluid.7-'0 A search of the literature revealed
only two cases similar to the one we have described. In the case reported by Thorp11 surgical treatment was chosen initially for a 26-year-old man with a carcinoma at the site of a lumbar meningomyelocele. Six months after resection the tumour recurred and was treated with radiotherapy. Later the tumour appeared again, necessitating further surgery. Three weeks postoperatively the patient died of septicemia. In the case described by Pope and Todorov1' a squamous cell carcinoma developed at the site of a cervical meningomyelocele in a 37-year-old man. This lesion was easily excised, without complications, because the defect was a meningocele and hence did not contain neural elements or connect with the spinal canal. Two cases of malignant teratomas
occurring in meningoceles and result-
FIG. 3-Tumour smaller and undergoing necrosis 8 weeks after completion of radiotherapy.
South Med J 58: 779, 1965 ing in death have also been re- 10. FERNHOFF PM, OAKLEY GP: Alphafetoprotein as predictor of fetal 19. LIDDELL K: Malignant change in ported.13'14 neural-tube defects. Lancet 2: 368, chronic varicose ulceration. PractiOne must be aware of possible 1975 tioner 215: 335, 1975 neoplastic change in untreated me- 11. THORP RH: Carcinoma associated 20. HALLIDAY JP: Squamous cell carciningomyeloceles after years of mewith myelomeningocele. Case report. noma in a venous ulcer. Med J A ust chanical irritation and chronic bacJ Neurosurg 27: 446, 1967 1: 449, 1968 terial infection. The defect, lacking 12. POPE M, ToDoRov AB: Cutaneous 21. BROWN HW, RIVERA J: Epidermoid a protective epithelial cover, must be squamous cell carcinoma as a rare carcinoma arising in a pilonidal sinus. complication of cervical meningocele. viewed regularly with a high degree Report of a case and review of the Birth Dejects 11: 336, 1975 of suspicion, as with other chronic literature. mt Surg 50: 435, 1968 ulcers; squamous cell carcinoma is a 13. LOVE JG: Delayed malignant devel- 22. DONSKY HJ, MENDELSON CG: Squamopment of a congenital teratoma with ous cell carcinoma as a complication well known complication of burn and spina bifida. Case report. J Neurosurg hidradenitis suppurativa. Arch of other .117 chronic venous ul29: 532, 1968 Dermatol 90: 488, 1964 cers,18'20 pilonidal sinuses,21 long- 14. MICKLE JP, MCLENNAN JE: Malignant 23. HUMPHREY PLAYFORm U, H, standing bacterial and fungal infecteratoma arising within a lipomeninLEAvELL UW Squamous JR: cell carcitions,'2.' vaccination scars26'27 and gocele. Case report. J Neurosurg 43: noma arising in hidradenitis suppura761, 1975 even tattoos.28 Once change is noted, tivum. Arch Dermatol 100: 59, 1969 biopsies should be done to establish 15. ARONS MS, LYNCH JB, LEWIS SR, et 24. FORSTROM L: Carcinomatous changes al: Scar tissue carcinoma: Part I. A a histologic diagnosis. If malignancy in lupus vulgaris. Ann Clin Res 1: clinical study with special reference is established, an intensive search for 213, 1969 to burn scar carcinoma. Ann Surg metastases, lymph node involvement 25. CAPLAN RM: Epidermoid carcinoma 161: 170, 1965 arising in extensive chromoblastomyand local invasion must be made for 16. BOSTwICK J iii, PENDERGRAST WJ, cosis. Arch Dermatol 97: 38, 1968 proper staging and subsequent treatVASCONEZ LO: Marjolin's ulcer: an MARMELZAT WL: Malignant tumors 26. ment. immunologically privileged tumor? in smallpox vaccination scars. Ibid, p Plast Reconstr Surg 57: 66, 1976 We thank Dr. Clair Williams for his 17. DIDOLKAR MS, GERNER RE, MOORE 400 permission to publish this case. 27. REED WB, WILsON-JONEs E: MaligGE: Epidermolysis bullosa dystronant tumors as late complication of phica and epithelioma of the skin: References vaccination. Arch Dermatol 98: 132, review of published cases and report 1. American Academy of Orthopedic Surgeons: Symposium on Myelomeningocele, Mosby, St. Louis, 1972, pp 1-20 2. FREEMAN JM: Practical Management of Meningomyelocele, Univ Park Pr,
of an additional patient. Cancer 33: 198, 1974 18. PENNELL TC, HIGHTOWER F: Malignant changes in post-phlebitic ulcers.
1968 28. MCQUARRIE DG: Squamous-cell carcinoma arising in a tattoo. Minn Med 49: 799, 1966
Baltimore, Md, 1974, pp 1-22
3. SMITh ED (ed): Spida Bitida and the Total Care of Spinal Myelomeningocele,
CC Thomas, Springfield, Ill,
Historical notes typhoid fever
1965, pp 3-46 4. HARRIS HW, MILLER OF: Midline
cutaneous and spinal defects. Arch Dermatol 112: 1724, 1976 5. HEMMER R: Meningoceles and myeloceles, in Progress in Neurological Surgery, vol 4, KRAYENBUHL H (ed), Phiebig, White Plains, NY, 1971, pp
192-226 6. SPECHT EE, GOODNER EK, TANAGHO EA, et al: Myelomeningocele: a symposium on orthopedic, ophthalmologic, urologic, psychological and social, neurosurgical and general considerations. West J Med 121: 281, 1974 7. BROCK DJH, SUTCLIFFE RG: Alphafetoprotein in the antenatal diagnosis of anencephaly and spina bifida. Lancet 2: 197, 1972 8. COLTART TM, SELLER MJ, SINGER
JD, et al: Amniotic fluid concentrations of alpha-fetoprotein (AEP) in early normal pregnancy, and pregnancy complicated by neural tube defects. A review of 18 months experience. Guy's Hasp Rep 123: 121, 1974 9. MILUNSKY A, ALPERT E: The value
of alpha-fetoprotein in the prenatal diagnosis of neural tube defects. J Pediatr 84: 889, 1974
In 1856 William Bud, a British small-town practitioner, published a series of articles in which he demonstrated that in the stools of patients with typhoid there was an agent that carried the disease to other patients (Dowling HF: Fighting Infection, Harvard University Press, Cambridge, Massachusetts, 1977). He evolved a system for preventing the spread of typhoid that included boiling contaminated linen, chemically disinfecting the discharges from typhoid patients and having attendants wash their hands. He considered that "the sewer may be looked upon... as a direct continuation of the diseased intestine" and urged that water and milk be boiled during an epidemic. Charles Murchison, a person of some prominence, dismissed these views and held that putrefaction was the cause of typhoid; Murchison's views prevailed for some time. In the early 1900s and until recently the greatest health problem
related to typhoid was deciding on the proper management of carriers of the disease. In 1906 George Soper of New York City's health departm.nt investigated six cases of typhoid that had occurred in one household. He learned that a recently employed Irish cook had left abruptly after a stay of 3 weeks. When he finally traced the cook he discovered that she had been the cause of seven epidemics of typhoid in 6 years. Her name was Mary Mallon, but she came to be known as Typhoid Mary. In all, she was responsible for approximately 60 cases of typhoid, 3 of which were fatal, in New York City. Once discovered, Mary was kept in hospital for 3 years, but she discharged herself and, against medical advice, took a job involving the handling of food; this led to a further series of epidemics. Cholecystectomy was the only known treatment for carriers at the time, but Mary would have no part of it; she spent the last 23 years of her life in hospital under what we today would call protective custody.u
CMA JOURNAL/OCTOBER 7, 1978/VOL. 119 741
