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A case of primary lymphosarcoma occupying two-thirds of the lumen of the stomach
A CASE OF PRIMARY LYMPHOSARCOMA OCCUPYING TWO/THIRDS OF THE LUMEN OF THE STOMACH SAUL ALFRED RITTER, PH.D., M.D., F.A.C.S. Assistant Attending Surgeon, Misericordia HospitaI NEW YORK CITY
I
T is generaIIy conceded that the incidence of primary gastric sarcoma is about I per cent of a11 gastric neoplasms. l,g*lo Pack and McNeerl in 1935 reported nine cases of sarcoma of the stomach, three of which were primary gastric Iymphosarcomata. They stated that out of 40,000 cases of benign and maIignant neopIasms of a11 organs treated at the MemoriaI HospitaI, there were 400 cases of maIignant neopIasms of the stomach. In that series there were onIy five cases of sarcoma, two of which were primary gastric Iymphosarcomata. They reported a third such case from the New Haven HospitaI. Cheever2 reviewed 628 cases of gastric tumors treated at the Peter Bent Brigham HospitaI, among which there were nine cases of Iymphosarcomata. Cases of genera1 Iymphosarcoma which have come to autopsy have not infrequentIy been found to have secondary metastatic invoIvement of the stomach. Two such cases were incIuded among those reported by Pack and McNeer. The first case of sarcoma of the stomach reported in the Iiterature was by SibIey in 1816, quoted by WaIton. Primary gastric Iymphosarcoma may occur at any age. FinIayson’s4 patient was 3j’i years oId. Jones and Carmody’Q patient was g years of age. Di Giacoma’s6 was 65 years oId, Gosset’s patient cited by BaIfour and McCann’ was aged 85 years. Sarcoma may grow from any aspect of the stomach. They have been described as intragastric, intramuraI, and even extragastric in Iocation. Types of gastric sarcoma reported in the Iiterature are angiosarcoma, spindIe-ceI1
sarcoma, neurosarcoma and fibrosarcoma. The Iatter two are of higher mahgnancy and occasionaIIy invoIve the pyIorus. Another type caIIed round or aIveoIar sarcoma is usuaIIy infiItrative in character and a Iess common variety. CIinicaIIy, it is most often impossibIe to differentiate between carcinoma and sarcoma of the stomach. Symptoms of epigastric pressure, pain, vomiting, hematemesis, tarry stooIs, anemia and Ioss of weight are common to both. Patients between 20 and 35 years of age or less, in whom a Iarge gastric neopIasm is found, most likely are suffering from sarcoma rather than carcinoma of the stomach. In addition to the usua1 Roentgen examination a gastroscopic examination shouId be made, preferabIy with the gastroscope containing the retrograde optica attachment described by Korbsch.8 This may aid in making a definite diagnosis. Very occasionaIIy a piece of the gastric neopIasm may become disIodged during regurgitation or whiIe doing a gastric anaIysis. PathoIogic examination of the piece of tissue wouId chnch the diagnosis. It is generaIIy recognized that when gastric carcinoma can be cIinicaIIy paIpated, it is most often aIready too late for surgica1 intervention because of metastases and even direct invasion of the surrounding structures. This is not necessariIy so in sarcoma of the stomach. Metastases, when they do occur, appear much Iater in the disease, and may be present in the perigastric Iymph nodes, liver, mesentery, Iungs, peritoneum and aIso in the superficia1 Iymph nodes. When the Iatter appear, a biopsy may easiIy confirm the diagnosis. ‘31
132
American Journal of Surgery
Ritter-Lymphosarcoma
The Lymphosarcoma is radiosensitive. maIignant Iymphocytes are sensitive to radium as we11 as to the Roentgen ray. Desjardins stated that this therapy aIone
JANUARY, IWO
reported surgicaIIy cured nineteen years after partial gastrectomy. This is the Iongest postoperative cure of this Iesion which has been reported.
Excised distal two-thirds of stomach showing tumor size of a baby’s head which grew from the posterior wall and occupied its Iumen.
FIG. I.
might cure earIy cases of gastric Iymphosarcoma. It wouId, however, appear to be a dangerous procedure to treat the Iarger tumors by radiation aIone because of possibIe necrosis, hemorrhage or perforation. Dranell advises the administration of medium wave Iength Roentgen therapy. Most cases reviewed in the Iiterature have not survived operation for more than one year. There have, however, been notabIe exceptions which have survived and have remained we11 for many years. BaIfour and McCann’s’ patients after gastrectomy Iived on the average of eIeven months and their Iongest surviva1 was nine years. Jones and Carmody’s6 case of gastric Iymphosarcoma in a boy g years oId, was
CASE
REPORT
M. T., 38 years of age, a Greek woman, was admitted to the Misericordia HospitaI on February 8, 1930. She was married and had three chiIdren. A choIecystectomy and appendectomy had been performed five years previously at another institution. For four months prior to admission to the hospita1 she had complained of epigastric cramp-like pains which radiated to the interscapular region. These occurred about twenty minutes after meals and Iasted for many hours. Nausea, vomiting and eructations were present. Fourteen days before admission she began to have hematemesis soon after taking food. This persisted for seven days. She had two simiIar attacks of copious bIoody vomiting during the ensuing week. The stooIs were tarry bIack in coIor, but there was no diarrhea. The patient had lost
NEW SERIES VOL. XLVII, No. I
Ritter-Lymphosarcoma
29 pounds within four months and was becoming progressively weaker. PhysicaI examination revealed the patient to be acutely ill. There was marked pallor and evidence of considerabIe loss of weight. The abdomen was protuberant. A Iarge tumor the size of a grape-fruit was easily paIpabIe in the left upper quadrant. It was tender and did not move with respirations, but seemed to be attached to the deeper structures. The mass was firm, irreguIar and extended behind the region of the spIeen. The Iiver was not enIarged and there was no ascites present. The heart and Iungs were cIinicaIIy negative. No enIarged Iymph nodes were palpable. The rectum and peIvic organs revealed no abnormaIity. Gastric analysis couId not be attempted in the presence of gross hematemesis and danger of perforation of the stomach. Roentgen ray examination revealed an unusua1 defect in the pars media and pars pylorica of the stomach. There was no evidence of obstruction. The roentgenoIogist could not be certain whether the tumor was intragastric or extragastric. CIinicaIIy, the Iesion was thought to be either carcinoma of the stomach or a pancreatic tumor. 3,670,ooo; Blood count showed Erythrocytes white ceI1.s 13,800; poIymorphonuclear Ieucocytes 80 per cent; Iymphocytes I 8 per cent; mononucIear ceIIs 2 per cent. The bIood pressure was I 16/80. BIood chemistry revealed: I; uric acid 3.4; urea nitrogen 15; creatinine sugar 106 mg. per cent. Cystoscopic examination and pyelography were negative. ExpIoratory operation was performed on February I 3, 1930. A median epigastric incision was made. Upon opening the peritoneum no free ffuid was encountered. The stomach waI1 was dilated and hypertrophied. An ova1 tumor the size of a baby’s head was found to be growing from the posterior waI1 of the stomach and occupying about two-thirds of its Iumen. A few metastases were present, only in the regiona Iymph nodes at the Iesser curvature. A subtota1 gastrectomy was performed. The upper third of the remaining part of the stomach was cIosed and a PoIya operation with retrocoIic anastomosis with the first portion of the jejunum was done. Moderate postoperative shock foIlowed, which was reheved with gIucose-saIine infusions and medication. A blood transfusion of 600 C.C. of whoIe blood was given.
American Journal of Surgery
I33
The patient raIIied after a stormy postoperative course. Soon after operation she was able to take smaI1 quantities of easiIy digestible
FIG. 2. Photomicrograph of the tumor showing round celIs varying in size containing large pale nucIei with numerous mitotic figures. The supporting tissue is scant, deIicate and contains few fibrits.
foods, and she became progressively stronger. She was discharged from the hospital on ApriI 20, 1930. Three weeks after she Ieft the hospital, I was caIIed hurriedly to see her during the night. She compIained of severe thoracic pains, cough and hemoptysis, and died dyspnea, suddenIy from what cIinicaIIy resembIed puImonary embolism. No permission for a postmortem examination could be obtained. PathoIogic examination of the portion of the stomach containing the tumor removed at operation was made by Dr. R. C. SchIeussner. The specimen (Fig. I) consisted of a section of stomach measuring 17 cm. X 20 cm. The stomach had been opened along the anterior wall and weighed 580 gm. Arising from the posterior waI1 was a large ovoid tumor 14 X 9 X 5 cm. It had a broad flat base and its surface was superficiaIIy uIcerated. On section it was of uniform grayish white coIor and was quite friabIe. The edge of the tumor could be seen to be infiItrating the waI1 of the stomach beneath the adjoining mucous membrane. Sections through the main body of the tumor showed that it was composed of round cells about IO mm. in diameter, with moderate variation in the size of the ceIIs. The nucIei
I34
American Journal of Surgery
Ritter-Lymphosarcoma
were Iarge and pale; numerous mitotic figures could be seen. The cytopIasm was scant and irregularly extended in pIaces. There was onIy a smaI1 amount of deIicate supporting tissue present, IittIe intraceIIuIar brown substance and very few fibrils. The tumor penetrated a11 coats of the stomach waI1 and dispIaced the norma tissue. Numerous tumor ceIIs infiItrated the stomach waI1 beneath the mucosa at the border of the tumor. The tumor tissue contained many thin waIIed bIood vesseIs. (Fig. 2.)
Patients with Iarge paIpabIe gastric tumors before middIe age shouId be suspected of having sarcoma of the stomach and not carcinoma. SubtotaI or even tota gastrectomy foIIowed by deep Roentgen therapy is the procedure generaIIy foIIowed. REFERENCES I.
SUMMARY
A case of primary lymphosarcoma occupying two-thirds of the Iumen of the stomach is reported. This occurred in a woman 38 years oId. Primary lymphosarcoma of the stomach occurs in about I per cent of a11 gastric neopIasms. The youngest patient with this Iesion reported in the Iiterature was 3% years of age, and the oIdest patient was 85 years. The longest surviva1 after gastrectomy reported surgicaIIy cured was 19 years (Jones and Carmody).
PACK, G. T., and MCNEER, G. P. Ann. Surg., IOI:
1206-1224 (May)
1935.
2. CHEEVER, D. Ann. Surg., 96: gI I-923
(Nov.) 1932. 2nd ed., 3. WALTON, A. J. Surgical Dyspepsias, London, 1930. Edward Arnold. 4. FINLAYSON,J. Brit. M. J. 2: 1535, 1899. 5. JONES, T. E., and CARMODY, M. G. Ann. Surg., IOI: 1136-1138 (April) 1935. 6. DI GIACOMA: Rijorma med., 31: 144-148 (Feb.) 19’5. 7. BALFOUR, D. C., and MCCANN J. C. Surg.,Gynec. IY Obst., 50: 948-953 (June) 1930. 8. KORBSCH, R. Deutscbe med. Wcbnscbr., 63: 14121416 (Sept.) 1937. and 9. EUSTERMAN and BALFOUR. The Stomach Duodenum. PhiIadelphia, 1936. W. B. Saunders. 10. EWING, J. Neoplastic Diseases. Philadelphia, 1928. W. B. Saunders. II. DRANE, R. Am. J. Roentgenol., 34: 755-758 (Dec.) 1935.
I
T is generaIIy conceded that the incidence of primary gastric sarcoma is about I per cent of a11 gastric neoplasms. l,g*lo Pack and McNeerl in 1935 reported nine cases of sarcoma of the stomach, three of which were primary gastric Iymphosarcomata. They stated that out of 40,000 cases of benign and maIignant neopIasms of a11 organs treated at the MemoriaI HospitaI, there were 400 cases of maIignant neopIasms of the stomach. In that series there were onIy five cases of sarcoma, two of which were primary gastric Iymphosarcomata. They reported a third such case from the New Haven HospitaI. Cheever2 reviewed 628 cases of gastric tumors treated at the Peter Bent Brigham HospitaI, among which there were nine cases of Iymphosarcomata. Cases of genera1 Iymphosarcoma which have come to autopsy have not infrequentIy been found to have secondary metastatic invoIvement of the stomach. Two such cases were incIuded among those reported by Pack and McNeer. The first case of sarcoma of the stomach reported in the Iiterature was by SibIey in 1816, quoted by WaIton. Primary gastric Iymphosarcoma may occur at any age. FinIayson’s4 patient was 3j’i years oId. Jones and Carmody’Q patient was g years of age. Di Giacoma’s6 was 65 years oId, Gosset’s patient cited by BaIfour and McCann’ was aged 85 years. Sarcoma may grow from any aspect of the stomach. They have been described as intragastric, intramuraI, and even extragastric in Iocation. Types of gastric sarcoma reported in the Iiterature are angiosarcoma, spindIe-ceI1
sarcoma, neurosarcoma and fibrosarcoma. The Iatter two are of higher mahgnancy and occasionaIIy invoIve the pyIorus. Another type caIIed round or aIveoIar sarcoma is usuaIIy infiItrative in character and a Iess common variety. CIinicaIIy, it is most often impossibIe to differentiate between carcinoma and sarcoma of the stomach. Symptoms of epigastric pressure, pain, vomiting, hematemesis, tarry stooIs, anemia and Ioss of weight are common to both. Patients between 20 and 35 years of age or less, in whom a Iarge gastric neopIasm is found, most likely are suffering from sarcoma rather than carcinoma of the stomach. In addition to the usua1 Roentgen examination a gastroscopic examination shouId be made, preferabIy with the gastroscope containing the retrograde optica attachment described by Korbsch.8 This may aid in making a definite diagnosis. Very occasionaIIy a piece of the gastric neopIasm may become disIodged during regurgitation or whiIe doing a gastric anaIysis. PathoIogic examination of the piece of tissue wouId chnch the diagnosis. It is generaIIy recognized that when gastric carcinoma can be cIinicaIIy paIpated, it is most often aIready too late for surgica1 intervention because of metastases and even direct invasion of the surrounding structures. This is not necessariIy so in sarcoma of the stomach. Metastases, when they do occur, appear much Iater in the disease, and may be present in the perigastric Iymph nodes, liver, mesentery, Iungs, peritoneum and aIso in the superficia1 Iymph nodes. When the Iatter appear, a biopsy may easiIy confirm the diagnosis. ‘31
132
American Journal of Surgery
Ritter-Lymphosarcoma
The Lymphosarcoma is radiosensitive. maIignant Iymphocytes are sensitive to radium as we11 as to the Roentgen ray. Desjardins stated that this therapy aIone
JANUARY, IWO
reported surgicaIIy cured nineteen years after partial gastrectomy. This is the Iongest postoperative cure of this Iesion which has been reported.
Excised distal two-thirds of stomach showing tumor size of a baby’s head which grew from the posterior wall and occupied its Iumen.
FIG. I.
might cure earIy cases of gastric Iymphosarcoma. It wouId, however, appear to be a dangerous procedure to treat the Iarger tumors by radiation aIone because of possibIe necrosis, hemorrhage or perforation. Dranell advises the administration of medium wave Iength Roentgen therapy. Most cases reviewed in the Iiterature have not survived operation for more than one year. There have, however, been notabIe exceptions which have survived and have remained we11 for many years. BaIfour and McCann’s’ patients after gastrectomy Iived on the average of eIeven months and their Iongest surviva1 was nine years. Jones and Carmody’s6 case of gastric Iymphosarcoma in a boy g years oId, was
CASE
REPORT
M. T., 38 years of age, a Greek woman, was admitted to the Misericordia HospitaI on February 8, 1930. She was married and had three chiIdren. A choIecystectomy and appendectomy had been performed five years previously at another institution. For four months prior to admission to the hospita1 she had complained of epigastric cramp-like pains which radiated to the interscapular region. These occurred about twenty minutes after meals and Iasted for many hours. Nausea, vomiting and eructations were present. Fourteen days before admission she began to have hematemesis soon after taking food. This persisted for seven days. She had two simiIar attacks of copious bIoody vomiting during the ensuing week. The stooIs were tarry bIack in coIor, but there was no diarrhea. The patient had lost
NEW SERIES VOL. XLVII, No. I
Ritter-Lymphosarcoma
29 pounds within four months and was becoming progressively weaker. PhysicaI examination revealed the patient to be acutely ill. There was marked pallor and evidence of considerabIe loss of weight. The abdomen was protuberant. A Iarge tumor the size of a grape-fruit was easily paIpabIe in the left upper quadrant. It was tender and did not move with respirations, but seemed to be attached to the deeper structures. The mass was firm, irreguIar and extended behind the region of the spIeen. The Iiver was not enIarged and there was no ascites present. The heart and Iungs were cIinicaIIy negative. No enIarged Iymph nodes were palpable. The rectum and peIvic organs revealed no abnormaIity. Gastric analysis couId not be attempted in the presence of gross hematemesis and danger of perforation of the stomach. Roentgen ray examination revealed an unusua1 defect in the pars media and pars pylorica of the stomach. There was no evidence of obstruction. The roentgenoIogist could not be certain whether the tumor was intragastric or extragastric. CIinicaIIy, the Iesion was thought to be either carcinoma of the stomach or a pancreatic tumor. 3,670,ooo; Blood count showed Erythrocytes white ceI1.s 13,800; poIymorphonuclear Ieucocytes 80 per cent; Iymphocytes I 8 per cent; mononucIear ceIIs 2 per cent. The bIood pressure was I 16/80. BIood chemistry revealed: I; uric acid 3.4; urea nitrogen 15; creatinine sugar 106 mg. per cent. Cystoscopic examination and pyelography were negative. ExpIoratory operation was performed on February I 3, 1930. A median epigastric incision was made. Upon opening the peritoneum no free ffuid was encountered. The stomach waI1 was dilated and hypertrophied. An ova1 tumor the size of a baby’s head was found to be growing from the posterior waI1 of the stomach and occupying about two-thirds of its Iumen. A few metastases were present, only in the regiona Iymph nodes at the Iesser curvature. A subtota1 gastrectomy was performed. The upper third of the remaining part of the stomach was cIosed and a PoIya operation with retrocoIic anastomosis with the first portion of the jejunum was done. Moderate postoperative shock foIlowed, which was reheved with gIucose-saIine infusions and medication. A blood transfusion of 600 C.C. of whoIe blood was given.
American Journal of Surgery
I33
The patient raIIied after a stormy postoperative course. Soon after operation she was able to take smaI1 quantities of easiIy digestible
FIG. 2. Photomicrograph of the tumor showing round celIs varying in size containing large pale nucIei with numerous mitotic figures. The supporting tissue is scant, deIicate and contains few fibrits.
foods, and she became progressively stronger. She was discharged from the hospital on ApriI 20, 1930. Three weeks after she Ieft the hospital, I was caIIed hurriedly to see her during the night. She compIained of severe thoracic pains, cough and hemoptysis, and died dyspnea, suddenIy from what cIinicaIIy resembIed puImonary embolism. No permission for a postmortem examination could be obtained. PathoIogic examination of the portion of the stomach containing the tumor removed at operation was made by Dr. R. C. SchIeussner. The specimen (Fig. I) consisted of a section of stomach measuring 17 cm. X 20 cm. The stomach had been opened along the anterior wall and weighed 580 gm. Arising from the posterior waI1 was a large ovoid tumor 14 X 9 X 5 cm. It had a broad flat base and its surface was superficiaIIy uIcerated. On section it was of uniform grayish white coIor and was quite friabIe. The edge of the tumor could be seen to be infiItrating the waI1 of the stomach beneath the adjoining mucous membrane. Sections through the main body of the tumor showed that it was composed of round cells about IO mm. in diameter, with moderate variation in the size of the ceIIs. The nucIei
I34
American Journal of Surgery
Ritter-Lymphosarcoma
were Iarge and pale; numerous mitotic figures could be seen. The cytopIasm was scant and irregularly extended in pIaces. There was onIy a smaI1 amount of deIicate supporting tissue present, IittIe intraceIIuIar brown substance and very few fibrils. The tumor penetrated a11 coats of the stomach waI1 and dispIaced the norma tissue. Numerous tumor ceIIs infiItrated the stomach waI1 beneath the mucosa at the border of the tumor. The tumor tissue contained many thin waIIed bIood vesseIs. (Fig. 2.)
Patients with Iarge paIpabIe gastric tumors before middIe age shouId be suspected of having sarcoma of the stomach and not carcinoma. SubtotaI or even tota gastrectomy foIIowed by deep Roentgen therapy is the procedure generaIIy foIIowed. REFERENCES I.
SUMMARY
A case of primary lymphosarcoma occupying two-thirds of the Iumen of the stomach is reported. This occurred in a woman 38 years oId. Primary lymphosarcoma of the stomach occurs in about I per cent of a11 gastric neopIasms. The youngest patient with this Iesion reported in the Iiterature was 3% years of age, and the oIdest patient was 85 years. The longest surviva1 after gastrectomy reported surgicaIIy cured was 19 years (Jones and Carmody).
PACK, G. T., and MCNEER, G. P. Ann. Surg., IOI:
1206-1224 (May)
1935.
2. CHEEVER, D. Ann. Surg., 96: gI I-923
(Nov.) 1932. 2nd ed., 3. WALTON, A. J. Surgical Dyspepsias, London, 1930. Edward Arnold. 4. FINLAYSON,J. Brit. M. J. 2: 1535, 1899. 5. JONES, T. E., and CARMODY, M. G. Ann. Surg., IOI: 1136-1138 (April) 1935. 6. DI GIACOMA: Rijorma med., 31: 144-148 (Feb.) 19’5. 7. BALFOUR, D. C., and MCCANN J. C. Surg.,Gynec. IY Obst., 50: 948-953 (June) 1930. 8. KORBSCH, R. Deutscbe med. Wcbnscbr., 63: 14121416 (Sept.) 1937. and 9. EUSTERMAN and BALFOUR. The Stomach Duodenum. PhiIadelphia, 1936. W. B. Saunders. 10. EWING, J. Neoplastic Diseases. Philadelphia, 1928. W. B. Saunders. II. DRANE, R. Am. J. Roentgenol., 34: 755-758 (Dec.) 1935.