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Meeting 12 November 1976
Cases posterior rectal mucosa, extending upwards beyond the reach of the examining finger. Routine hamatological investigations and chest X-ray were normal, but X-rays of the pelvis revealed a destructive lesion in the sacrum extending downW O'N, man aged 53, security officer wards from the lower border of S2. The appearHistory: Presented in November 1975 with a one- ances were best seen on the lateral film, which year history of discomfort over the sacrum, latterly showed a large overlying soft-tissue mass in the with a feeling of incomplete and painful defeca- presacral soft tissues displacing the rectum fortion. There was no history of trauma to the spine, wards (Fig IA, B). A provisional diagnosis of chordoma was conproblems with micturition or sexual performance, but occasionally he experienced radiation of pain firmed by biopsy of the sacral swelling. Histology to his right thigh. The patient gave no relevant showed a neoplasm consisting of cell cords and previous history and was otherwise quite well. lobules embedded in myxoid matrix (Fig 2). A Examination: The only clinical finding was a tender large number of the cells had a vacuolated cytovisible smooth swelling over the sacrum. Rectal plasm with hyperchromatic nuclei, the pattern examination revealed a firm mass approximately being that of 'physaliferous' cells characteristic of a 7 cm in diameter, behind but not attached to the chordoma. Sacrococcygeal Chordoma J E Hale MS FRCS (for P Aichroth MS FRCS and A G Rutter FRCS)
Fig IA, B X-rays (10.12.75) showing a destructive lesion in the sacrum extending downwardsfrom S2. On the lateral film a soft-tissue mass in the presacral area displaces the rectum forwards
Clinical Section
277
Fig 2 Photomicrograph ofchordoma showing 'physaliferous' cells with vacuolated cytoplasm and hyperchromatic nuclei (H & E x 16)
Operation (17 December 1975): The tumour was approached through a posterior vertical midline incision extending from L3 to the anococcygeal raphe which was divided, the coccyx removed and the rectum separated from the lesion by blunt dissection. The lumbosacral junction was exposed and the laminae of the upper two sacral vertebrae removed to reveal a soft friable tumour arising from the second vertebra and invading the sacral canal. The dura was opened and the sacrum divided at the level of the second foramina. The upper three nerve roots were dissected from the tumour and preserved except for the right S3 root, which was damaged. The distal sacrum with the tumour attached was removed, the dura repaired, a drain inserted and the wound closed. Recovery was satisfactory with normal control of sphincters, but on the eleventh day after operation he developed an E. coli meningitis which responded to parenteral and intrathecal antibiotics, including gentamicin. The patient then experienced profuse diarrhoea due to pseudomembranous colitis, which improved once antibiotics were discontinued. A collection of cerebrospinal fluid beneath the scar was aspirated, and three months after operation he was discharged. One year later he remains well with no clinical or radiological evidence of recurrence, and he has normal sphincter control and sexual function.
Discussion The chordoma is a tumour that occurs rarely and it originates from notochord remnants at either end of the spinal axis. Since the first sacrococcygeal chordoma was reported (Hennig 1900) there have been a number of reviews of chordomas, including 505 cases reviewed by Utne & Pugh in 1955, of which 39 % were cranial, 16 % were vertebral and 45 % were sacrococcygeal. In a comprehensive review of 222 patients with sacrococcygeal chordoma (Gray et al. 1975) it was noted that after the age of 40, men are affected twice as often as women. Sacrococcygeal chordomas have to be differentiated from other retrorectal lesions such as a pararectal abscess and myeloma deposits (Freier et al. 1971) The diagnosis is often delayed because of an insidious onset and a variety of symptoms, the commonest of which is pain due to mechanical pressure. Involvement of the sacral plexus may produce pain radiating to the buttocks and perineum or along the distribution of one or both sacral nerves. Saddle anesthesia with rectal and bladder dysfunction may occur and increasing constipation due to forward displacement of the rectum frequently occurs, as in this case. The distinctive radiological appearances best seen on lateral films include anterior expansion of the sacrum, rarefaction or destruction of bone and
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sometimes calcification of necrotic tumour (Hsieh & Hsieh 1936). Metastatic spread occurs in only 10% of cases and the condition is usually fatal because of local invasion. Treatment by complete surgical removal offers the best chance of cure (MacCarty et al. 1961), but resectability is often limited by involvement of the sacral plexus, the upper nerve roots of which must be preserved to maintain sphincter control. Radiotherapy has been recommended where complete excision is not possible (Pearlman & Friedman 1970), and it is useful for palliation of pain due to recurrent disease, which may be delayed for many years (Sennett 1953).
Table I Investigations ESR varied between 26 and 56 mm (Westergren) in the 1st hour Chest X-ray, IVP, liver function tests, white cell count, urea and electrolytes were all normal Barium meal and follow-through showed a large duodenal diverticulum but no other pathology Latex test, ANF, LE cells and stool for examination and culture were all negative Agglutination tests for salmonella and brucella, 1: 20 Toxoplasma dye test negative Cytomegalovirus CF antibody titre, 1: 32 BSP excretion test: 5 % dye retained after 45 min Liver biopsy failed Sternal marrow normal Lymphangiogram: non-specific normal appearances No acid-fast bacilli seen or grown from urine or sputum Laparotomy was carried out on 5 February 1975
REFERENCES Freier D T, Stanley J C & Thompson N W (1971) Surgery, Gynecology and Obstetrics 132, 681-686 Gray S W, Singhabhandhu B, Smith R A & Skandalakis J E (1975) Surgery 78, 573-582 Hennig L (1900) Beitrage zur pathologischen Anatomie und zur allgemeinen Pathologie 28, 593 Hsieh C K & Hsieh H H (1936) Radiology 27, 101 MacCarty C S, Waugh J M & Coventry M B (1961) Surgery, Gynecology and Obstetrics 113, 551-554 Pearhnan A W & Friedman M (1970) American Journal of Roentgenology 108, 333-341 Sennett E J (1953) American Journal of Roentgenology 69, 613-622 Utne & Pugh (1955) American Journal of Roentgenology 74, 593
rheumatic mitral valve disease and had previously been treated for infective endocarditis, due to Streptococcus viridans, in 1953. On admission he was pyrexial (38°C) with splenomegaly, and gave a history of night sweats and Weight loss. His hemoglobin fell from 11.5 to 9.7 %. Seven successive blood cultures grew a diphtheroid species, which was also grown from skin swab cultures. The organism was identified by the National Collection of Type Cul-tures at Colindale as a lactobacillus, and dismissed as a contaminant. Multiple investigations (see Table 1) to find an alternative etiology were undertaken, but all proved negative. Eventually laparotomy was performed, proceeding to splenectomy, in the expecLactobacillus Infective Endocarditis tation of finding a lymphoma. David Isenberg MRCP Histology, however, showed a normal spleen (for A B S Mitchell MRCP) apart from one infarct (Fig 1). A postoperative (St Ann's Hospital, Tottenham, London N15) urinary tract infection was treated with a two-week course of ampicillin. A S, man aged 50 His pyrexia and night sweats settled and he was This patient, who worked as a clerical officer for the Post Office, was admitted into the Prince of discharged; remaining well until early 1976, when Wales's Hospital in November 1974 with suspected infective endocarditis. He was known to have Table 2 Classification of lactobacilli (Bergey 1957) (1) Homofermentative (producing lactic acid only from glucose) (2) Heterofermentative (producing other end-products such as CO2, alcohol and acetic acid, besides lactic acid, from glucose)
Fig 1 Splenic infraction (small circle shown is an artifact)
Optimum temperature 37-60C L. caucalasicus L. lactis L. helveticus L. acidophilus L. bifidus L. bulgarius L. delbrueckii
Optimum temperature 28-320C L. pastorianus
Optimum temperature 28-320C L. casei L. Ieichannii L. plantarum
Optimum temperature 35-40°C L. fermenti
L. buchner L. brevis
Proc. roy. Soc. Med. Volume 70 April 1977
Meeting 12 November 1976
Cases posterior rectal mucosa, extending upwards beyond the reach of the examining finger. Routine hamatological investigations and chest X-ray were normal, but X-rays of the pelvis revealed a destructive lesion in the sacrum extending downW O'N, man aged 53, security officer wards from the lower border of S2. The appearHistory: Presented in November 1975 with a one- ances were best seen on the lateral film, which year history of discomfort over the sacrum, latterly showed a large overlying soft-tissue mass in the with a feeling of incomplete and painful defeca- presacral soft tissues displacing the rectum fortion. There was no history of trauma to the spine, wards (Fig IA, B). A provisional diagnosis of chordoma was conproblems with micturition or sexual performance, but occasionally he experienced radiation of pain firmed by biopsy of the sacral swelling. Histology to his right thigh. The patient gave no relevant showed a neoplasm consisting of cell cords and previous history and was otherwise quite well. lobules embedded in myxoid matrix (Fig 2). A Examination: The only clinical finding was a tender large number of the cells had a vacuolated cytovisible smooth swelling over the sacrum. Rectal plasm with hyperchromatic nuclei, the pattern examination revealed a firm mass approximately being that of 'physaliferous' cells characteristic of a 7 cm in diameter, behind but not attached to the chordoma. Sacrococcygeal Chordoma J E Hale MS FRCS (for P Aichroth MS FRCS and A G Rutter FRCS)
Fig IA, B X-rays (10.12.75) showing a destructive lesion in the sacrum extending downwardsfrom S2. On the lateral film a soft-tissue mass in the presacral area displaces the rectum forwards
Clinical Section
277
Fig 2 Photomicrograph ofchordoma showing 'physaliferous' cells with vacuolated cytoplasm and hyperchromatic nuclei (H & E x 16)
Operation (17 December 1975): The tumour was approached through a posterior vertical midline incision extending from L3 to the anococcygeal raphe which was divided, the coccyx removed and the rectum separated from the lesion by blunt dissection. The lumbosacral junction was exposed and the laminae of the upper two sacral vertebrae removed to reveal a soft friable tumour arising from the second vertebra and invading the sacral canal. The dura was opened and the sacrum divided at the level of the second foramina. The upper three nerve roots were dissected from the tumour and preserved except for the right S3 root, which was damaged. The distal sacrum with the tumour attached was removed, the dura repaired, a drain inserted and the wound closed. Recovery was satisfactory with normal control of sphincters, but on the eleventh day after operation he developed an E. coli meningitis which responded to parenteral and intrathecal antibiotics, including gentamicin. The patient then experienced profuse diarrhoea due to pseudomembranous colitis, which improved once antibiotics were discontinued. A collection of cerebrospinal fluid beneath the scar was aspirated, and three months after operation he was discharged. One year later he remains well with no clinical or radiological evidence of recurrence, and he has normal sphincter control and sexual function.
Discussion The chordoma is a tumour that occurs rarely and it originates from notochord remnants at either end of the spinal axis. Since the first sacrococcygeal chordoma was reported (Hennig 1900) there have been a number of reviews of chordomas, including 505 cases reviewed by Utne & Pugh in 1955, of which 39 % were cranial, 16 % were vertebral and 45 % were sacrococcygeal. In a comprehensive review of 222 patients with sacrococcygeal chordoma (Gray et al. 1975) it was noted that after the age of 40, men are affected twice as often as women. Sacrococcygeal chordomas have to be differentiated from other retrorectal lesions such as a pararectal abscess and myeloma deposits (Freier et al. 1971) The diagnosis is often delayed because of an insidious onset and a variety of symptoms, the commonest of which is pain due to mechanical pressure. Involvement of the sacral plexus may produce pain radiating to the buttocks and perineum or along the distribution of one or both sacral nerves. Saddle anesthesia with rectal and bladder dysfunction may occur and increasing constipation due to forward displacement of the rectum frequently occurs, as in this case. The distinctive radiological appearances best seen on lateral films include anterior expansion of the sacrum, rarefaction or destruction of bone and
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Proc. roy. Soc. Med. Volume 70 April 1977
sometimes calcification of necrotic tumour (Hsieh & Hsieh 1936). Metastatic spread occurs in only 10% of cases and the condition is usually fatal because of local invasion. Treatment by complete surgical removal offers the best chance of cure (MacCarty et al. 1961), but resectability is often limited by involvement of the sacral plexus, the upper nerve roots of which must be preserved to maintain sphincter control. Radiotherapy has been recommended where complete excision is not possible (Pearlman & Friedman 1970), and it is useful for palliation of pain due to recurrent disease, which may be delayed for many years (Sennett 1953).
Table I Investigations ESR varied between 26 and 56 mm (Westergren) in the 1st hour Chest X-ray, IVP, liver function tests, white cell count, urea and electrolytes were all normal Barium meal and follow-through showed a large duodenal diverticulum but no other pathology Latex test, ANF, LE cells and stool for examination and culture were all negative Agglutination tests for salmonella and brucella, 1: 20 Toxoplasma dye test negative Cytomegalovirus CF antibody titre, 1: 32 BSP excretion test: 5 % dye retained after 45 min Liver biopsy failed Sternal marrow normal Lymphangiogram: non-specific normal appearances No acid-fast bacilli seen or grown from urine or sputum Laparotomy was carried out on 5 February 1975
REFERENCES Freier D T, Stanley J C & Thompson N W (1971) Surgery, Gynecology and Obstetrics 132, 681-686 Gray S W, Singhabhandhu B, Smith R A & Skandalakis J E (1975) Surgery 78, 573-582 Hennig L (1900) Beitrage zur pathologischen Anatomie und zur allgemeinen Pathologie 28, 593 Hsieh C K & Hsieh H H (1936) Radiology 27, 101 MacCarty C S, Waugh J M & Coventry M B (1961) Surgery, Gynecology and Obstetrics 113, 551-554 Pearhnan A W & Friedman M (1970) American Journal of Roentgenology 108, 333-341 Sennett E J (1953) American Journal of Roentgenology 69, 613-622 Utne & Pugh (1955) American Journal of Roentgenology 74, 593
rheumatic mitral valve disease and had previously been treated for infective endocarditis, due to Streptococcus viridans, in 1953. On admission he was pyrexial (38°C) with splenomegaly, and gave a history of night sweats and Weight loss. His hemoglobin fell from 11.5 to 9.7 %. Seven successive blood cultures grew a diphtheroid species, which was also grown from skin swab cultures. The organism was identified by the National Collection of Type Cul-tures at Colindale as a lactobacillus, and dismissed as a contaminant. Multiple investigations (see Table 1) to find an alternative etiology were undertaken, but all proved negative. Eventually laparotomy was performed, proceeding to splenectomy, in the expecLactobacillus Infective Endocarditis tation of finding a lymphoma. David Isenberg MRCP Histology, however, showed a normal spleen (for A B S Mitchell MRCP) apart from one infarct (Fig 1). A postoperative (St Ann's Hospital, Tottenham, London N15) urinary tract infection was treated with a two-week course of ampicillin. A S, man aged 50 His pyrexia and night sweats settled and he was This patient, who worked as a clerical officer for the Post Office, was admitted into the Prince of discharged; remaining well until early 1976, when Wales's Hospital in November 1974 with suspected infective endocarditis. He was known to have Table 2 Classification of lactobacilli (Bergey 1957) (1) Homofermentative (producing lactic acid only from glucose) (2) Heterofermentative (producing other end-products such as CO2, alcohol and acetic acid, besides lactic acid, from glucose)
Fig 1 Splenic infraction (small circle shown is an artifact)
Optimum temperature 37-60C L. caucalasicus L. lactis L. helveticus L. acidophilus L. bifidus L. bulgarius L. delbrueckii
Optimum temperature 28-320C L. pastorianus
Optimum temperature 28-320C L. casei L. Ieichannii L. plantarum
Optimum temperature 35-40°C L. fermenti
L. buchner L. brevis
