- Email: [email protected]
Multiple leiomyosarcomas of the jejunum with metastasis to inguinal lymph nodes
Multiple Leiomyosarcomas with Metastasis to Inguinal
of the Jejunum Lymph Nodes
T. K. DAS GUPTA, M.D., LEONARD H. KOLB, M.D. AND JOHN G. GRUHN, Chicago, Illinois From tbe Departments of Surgery and Patbology, Mount Sinai Hospital, and tbe Division of Oncology, Tbe Chicago Medical School, Chicago, Illinois.
M.D.,
of metastasis to the lymph nodes from a Ieiomyosarcoma in the jejunum. The case is also unusua1 in that the patient was admitted because of a mass in the right side of the groin which was thought to be a hernia or a lymph node enlargement of an undetermined type, but he did not have gastrointestinal symptoms. The mass was biopsied and found to be a metastatic Ieiomyosarcoma; subsequent radiographic examinations for a primary site reveaIed muItipIe sources within the jejunum.
EIOMYOSARCOMA of the smaI1 intestine is uncommon in the experience of any singIe surgeon. NevertheIess, by 1955 Starr and 230 reported cases of Dockerty [r] surveyed myomatous tumors of the smaII intestine and added seventy-six cases of their own from the Mayo Clinic. In over 60 per cent of the latter cases, the Iesion was malignant. In previous reviews, the emphasis has been on the frequency with which these tumors ulcerate and bleed, the extensive necrosis which they undergo and the incidence of mahgnancy [2]. The occurrence of metastasis to the Iymph nodes is extremely rare. Starr and Dockerty reviewed four cases in which there were metastases in the Iymph nodes but faiIed to find any iIIustrated histologic documentation. In view of this observation, they and others advised that surgica1 management of leiomyosarcoma consist of adequate resection of the lesion and the surrounding tissue without attempting to resect the regiona nodes and reIated Iymphatics. More recently, Darling and Welch [?I, in a review of eight cases of Ieiomyosarcoma of the smaII boweI at Massachusetts General Hospital from 1913 to 1957, found only one patient in whom the Iymph nodes were involved and this was by direct extension. Humphreys [4] recahed an unreported case of an inguinal metastasis from a gastric Ieiomyosarcoma which occurred fourteen years after the diagnosis was first established. The relative raritv of the disease itseIf, the infrequency of m&pIe primary sites and the Iack of an illustrated report of metastasis to the Iymph nodes warrants the following case report. To the best of our knowledge this is the first i1Iustrated case
L
CASE REPORT A seventy-two year old white man was admitted to Mount Sinai Hospital on March 20, 1~60, with a painIess nontender inguinal mass on the right side which had been apparent and growing slowly in size for four months. There was no evidence of Ioss of weight. PhysicaI examination of the right inguinal region revealed a mass 6 by 4 cm., oval in shape, over the fossa ovalis. In addition, the liver with a soft sharp edge was palpable two lingcrbreadths below the costal margin. Neither spleno-
FIG. I. Cut surface of a lymph node which has been replaced largely by fleshy tumorous tissue. 861
American
Journal
of Surgery,
Volume
102,
December
1961
Das Gupta, KoIb and Gruhn
2A FIG. 2. Roentgenograms
2B
showing the fiIIing defect in the proxima1 portion
(A) and the dista1 portion
(B) of the
jejunum. megaly nor any other detectabIe Iymphadenopathy was present. On admission, the laboratory studies discIosed 10.1 gm. per cent; the folIowing: hemogIobin, 30.5 per cent; red bIood ceI1 count, 3.83 hematocrit, miIIion per cu. mm.; white bIood cel1 count, 8.650 per cu. mm.; and the differentia1 bIood count was within norma limits. The results of urinaIysis, of analysis of the serum and of roentgenographic examination of the chest and the determinations of blood sugar and bIood urea were noncontributory. The inguina1 mass was excised on March 22, 1960. The specimen consisted of two Iymph nodes; one measured 5.5 by 3.5 by 2.5 cm., and the other was 3 by 2.5 by 2.5 cm. ExternalIy the capsule was intact. Cut sections discIosed a buIging surface which appeared tan and fleshy. (Fig. I.) Microscopic sections demonstrated a periphera1 rim of Iymphoid tissue but the buIk of the node was replaced by interlacing bundIes of spindle-shaped ceIIs with hyperchromatic moderateIypIeomorphic bIunt end nucIei. Moderate numbers of mitotic figures were present in every fieId. Van Gieson’s and Masson’s stains discIosed IittIe fibrous tissue. The Snook siIver stain demonstrated a fine reticuIin pattern between individual tumor cells. Phosphotungstic acid hematoxylin stain on Zenker-fixed tissue demonstrated myofibriIs. The pathoIogic diagnosis was metastatic Ieiomyosarcoma in the right inguina1 Iymph nodes.* The patient was readmitted as soon thereafter as feasibIe to estabIish the primary site. He had been essentiaIIy asymptomatic at home. A second physicaI examination discIosed no additiona reIevant * Diagnostic consuItation with Dr. phreys, BiIIings HospitaI, University appreciativeIy acknowIedged.
findings save for the we11 healed scar in the right side of the groin. Further laboratory studies included repeated determinations of hemoglobin; the resuIts persistentIy approximated IO gm. per cent indicating miId iron-deficiency anemia. The stoo1 was positive for occuIt bIood on three occasions during the time the patient was on a meat-free diet. The resuIts of anaIyses for bIood sugar, blood urea nitrogen, serum electrolytes, serum proteins, aIbumin and gIobuIin, protein eIectrophoresis and of studies of Iiver function, bone marrow aspiration, serum carotene and serum enzymes were within norma Iimits. RoentgenoIogic studies of the upper gastrointestina1 tract reveaIed two areas of filling defect, one in the proxima1 portion of the jejunum and the other in the dista1 part of the jejunum. (Fig. 2A and B.) Repeated examinations indicated this fiIIing defect was persistent. The results of Iower gastrointestina1 studies were negative. After the primary sites of Ieiomyosarcomas were roentgenographicaIIy demonstrated in the jejunum, an expIoratory operation was performed. One mass was found in the proximal portion of the jejunum 1.5 inches from the Iigament of Treitz about and two similar masses were present in the distal part of the jejunum. No other gross site of disease was noted. The Iiver and spIeen were normaI, and the pre- and para-aortic Iymph nodes were free of metastases on inspection and palpation. A wide segmenta resection of the jejunum, incIuding its mesentery, was performed. Intestinal continuity was established with an end to end anastomosis. Biopsy of the liver was also performed. The patient’s postoperative course was without comphcations and he was discharged tweIve days
EIeanor Humof Chicago, is
862
MuItipIe
Leiomyosarcomas
of the Jejunum
FIG. 3. Three foci of Ieiomyosarcoma in the jejunum. 1 .arge unopened segment includes two eIlipsoidal tumor masses, small opcncd portion includes a third tumor ,nass.
FIG. 4. Surface of predominantly intramural tumors the jcjunum is focally hemorrhagic and uiccratcd.
FIG. 5. Mid-power photomicrograph of Ieiomyosarcoma showing typica pattern from node. The same pattern was demonstrated in tumorous tissue taken from the jcjunum. (Original mngnifrcation X 120.)
FIG. 6. Migh power photomicrograph Ieiomyosarcoma showing typical pattern in jejunum. The same pattern was demonstrated in tumorous tissue taken from nodes. (Original magnification X 480.1
later. The patient was on a reguIar diet at the time of discharge. During pathologic examination of the specimen obtained during the operation (Figs. 3 and 4), the small finger of the examiner could bareIy be passed through the partially occIuded lumen at the tumor sites. The cut section of the tumor demonstrated firm tan fIeshy tissue predominantly intramural, extending from the Iuminal surface throughout the entire wal1. The luminal surface above the tumor was ulcerated, hemorrhagic and focally covered with necrotic debris. The serosa was not penetrated by the tumor. The mesenteric lymph nodes were free of tumorous tissue. HistoIogic sections of the leiomyosarcoma of the jejunum were precisely
similar
to the
metastases
the inguinal lymph nodes. biopsy of the liver disclosed
in
previously
observed in (Figs. 5 and 6.) The normal hrpatic tissue.
COMMENTS
In this case, several interesting features were demonstrated. Since the patient had a mass in the right side of the groin at the outset without gastrointestina1 symptoms, the possibiIity of a tumor in the smaI1 bowe1 was not conskered unti1 after excision biopsy of the node. The persistent resuIts of tests for occult bIood in the stoo1 and the hemoglobin value of 863
Das Gupta,
KoIb and Gruhn SUMMARY
10.1 gm. per cent directed our attention to a bIeeding source in the gastrointestina1 tract. Intestinal bleeding has been present in 53 per cent of symptomatic patients [5]. Moreover, pathoIogic examination of the specimen revealed an impending obstruction which occurs in 70 to 76 per cent of patients. Roentgenologic examination of the upper gastrointestinal tract has provided previousIy a correct preoperative diagnosis of a tumor in the smaII bowel in 84 per cent of the patients. MuItipIicity of intestina1 sites of Ieiomyosarcoma is uncommon. OnIy two of the fortyone cases reported by Starr and Dockerty were multipIe [I]. In the instance reported herein, the Iesions appeared autochthonous rather than metastatic within the jejunum. The rare occurrence of Iymph node metastases from Ieiomyosarcoma of the smaII bowe1 shouId not be dismissed entireIy when pIanning surgica1 excision of these tumors.
A case of Ieiomyosarcomas of the jejunum of a patient with obvious Iymph node metastases in the right side of the groin is reported. This is the first reported case of Ieiomyosarcomas of the jejunum metastasizing to inguina1 lymph nodes. REFERENCES I. STARR, G. F. and DOCKERTY, M.
B. Leiomyomas and Ieiomyosarcomas of the small intestine. Cancer, 8: 101, 1955. z. HORSELY. W. G. and MEANS. L. R. Leiomvosarcoma of the jejunum, with two case reports and a review of the literature. Ann. Surg., 141: 7999 1955. 3. DARLING, R. C. and WELCH, C. E. Tumors of the smaI1 intestine. New England J. Med., 260: 397, 1959. 4. HIJMPHREYS, E. M. Personal communication, 1960. 5. WEINSTEIN, M. and ROBERTS, M. Leiomyosarcoma of the duodenum. Report of a case and summary of the literature. Arch. Surg., 66: 318, 1953.
864
of the Jejunum Lymph Nodes
T. K. DAS GUPTA, M.D., LEONARD H. KOLB, M.D. AND JOHN G. GRUHN, Chicago, Illinois From tbe Departments of Surgery and Patbology, Mount Sinai Hospital, and tbe Division of Oncology, Tbe Chicago Medical School, Chicago, Illinois.
M.D.,
of metastasis to the lymph nodes from a Ieiomyosarcoma in the jejunum. The case is also unusua1 in that the patient was admitted because of a mass in the right side of the groin which was thought to be a hernia or a lymph node enlargement of an undetermined type, but he did not have gastrointestinal symptoms. The mass was biopsied and found to be a metastatic Ieiomyosarcoma; subsequent radiographic examinations for a primary site reveaIed muItipIe sources within the jejunum.
EIOMYOSARCOMA of the smaI1 intestine is uncommon in the experience of any singIe surgeon. NevertheIess, by 1955 Starr and 230 reported cases of Dockerty [r] surveyed myomatous tumors of the smaII intestine and added seventy-six cases of their own from the Mayo Clinic. In over 60 per cent of the latter cases, the Iesion was malignant. In previous reviews, the emphasis has been on the frequency with which these tumors ulcerate and bleed, the extensive necrosis which they undergo and the incidence of mahgnancy [2]. The occurrence of metastasis to the Iymph nodes is extremely rare. Starr and Dockerty reviewed four cases in which there were metastases in the Iymph nodes but faiIed to find any iIIustrated histologic documentation. In view of this observation, they and others advised that surgica1 management of leiomyosarcoma consist of adequate resection of the lesion and the surrounding tissue without attempting to resect the regiona nodes and reIated Iymphatics. More recently, Darling and Welch [?I, in a review of eight cases of Ieiomyosarcoma of the smaII boweI at Massachusetts General Hospital from 1913 to 1957, found only one patient in whom the Iymph nodes were involved and this was by direct extension. Humphreys [4] recahed an unreported case of an inguinal metastasis from a gastric Ieiomyosarcoma which occurred fourteen years after the diagnosis was first established. The relative raritv of the disease itseIf, the infrequency of m&pIe primary sites and the Iack of an illustrated report of metastasis to the Iymph nodes warrants the following case report. To the best of our knowledge this is the first i1Iustrated case
L
CASE REPORT A seventy-two year old white man was admitted to Mount Sinai Hospital on March 20, 1~60, with a painIess nontender inguinal mass on the right side which had been apparent and growing slowly in size for four months. There was no evidence of Ioss of weight. PhysicaI examination of the right inguinal region revealed a mass 6 by 4 cm., oval in shape, over the fossa ovalis. In addition, the liver with a soft sharp edge was palpable two lingcrbreadths below the costal margin. Neither spleno-
FIG. I. Cut surface of a lymph node which has been replaced largely by fleshy tumorous tissue. 861
American
Journal
of Surgery,
Volume
102,
December
1961
Das Gupta, KoIb and Gruhn
2A FIG. 2. Roentgenograms
2B
showing the fiIIing defect in the proxima1 portion
(A) and the dista1 portion
(B) of the
jejunum. megaly nor any other detectabIe Iymphadenopathy was present. On admission, the laboratory studies discIosed 10.1 gm. per cent; the folIowing: hemogIobin, 30.5 per cent; red bIood ceI1 count, 3.83 hematocrit, miIIion per cu. mm.; white bIood cel1 count, 8.650 per cu. mm.; and the differentia1 bIood count was within norma limits. The results of urinaIysis, of analysis of the serum and of roentgenographic examination of the chest and the determinations of blood sugar and bIood urea were noncontributory. The inguina1 mass was excised on March 22, 1960. The specimen consisted of two Iymph nodes; one measured 5.5 by 3.5 by 2.5 cm., and the other was 3 by 2.5 by 2.5 cm. ExternalIy the capsule was intact. Cut sections discIosed a buIging surface which appeared tan and fleshy. (Fig. I.) Microscopic sections demonstrated a periphera1 rim of Iymphoid tissue but the buIk of the node was replaced by interlacing bundIes of spindle-shaped ceIIs with hyperchromatic moderateIypIeomorphic bIunt end nucIei. Moderate numbers of mitotic figures were present in every fieId. Van Gieson’s and Masson’s stains discIosed IittIe fibrous tissue. The Snook siIver stain demonstrated a fine reticuIin pattern between individual tumor cells. Phosphotungstic acid hematoxylin stain on Zenker-fixed tissue demonstrated myofibriIs. The pathoIogic diagnosis was metastatic Ieiomyosarcoma in the right inguina1 Iymph nodes.* The patient was readmitted as soon thereafter as feasibIe to estabIish the primary site. He had been essentiaIIy asymptomatic at home. A second physicaI examination discIosed no additiona reIevant * Diagnostic consuItation with Dr. phreys, BiIIings HospitaI, University appreciativeIy acknowIedged.
findings save for the we11 healed scar in the right side of the groin. Further laboratory studies included repeated determinations of hemoglobin; the resuIts persistentIy approximated IO gm. per cent indicating miId iron-deficiency anemia. The stoo1 was positive for occuIt bIood on three occasions during the time the patient was on a meat-free diet. The resuIts of anaIyses for bIood sugar, blood urea nitrogen, serum electrolytes, serum proteins, aIbumin and gIobuIin, protein eIectrophoresis and of studies of Iiver function, bone marrow aspiration, serum carotene and serum enzymes were within norma Iimits. RoentgenoIogic studies of the upper gastrointestina1 tract reveaIed two areas of filling defect, one in the proxima1 portion of the jejunum and the other in the dista1 part of the jejunum. (Fig. 2A and B.) Repeated examinations indicated this fiIIing defect was persistent. The results of Iower gastrointestina1 studies were negative. After the primary sites of Ieiomyosarcomas were roentgenographicaIIy demonstrated in the jejunum, an expIoratory operation was performed. One mass was found in the proximal portion of the jejunum 1.5 inches from the Iigament of Treitz about and two similar masses were present in the distal part of the jejunum. No other gross site of disease was noted. The Iiver and spIeen were normaI, and the pre- and para-aortic Iymph nodes were free of metastases on inspection and palpation. A wide segmenta resection of the jejunum, incIuding its mesentery, was performed. Intestinal continuity was established with an end to end anastomosis. Biopsy of the liver was also performed. The patient’s postoperative course was without comphcations and he was discharged tweIve days
EIeanor Humof Chicago, is
862
MuItipIe
Leiomyosarcomas
of the Jejunum
FIG. 3. Three foci of Ieiomyosarcoma in the jejunum. 1 .arge unopened segment includes two eIlipsoidal tumor masses, small opcncd portion includes a third tumor ,nass.
FIG. 4. Surface of predominantly intramural tumors the jcjunum is focally hemorrhagic and uiccratcd.
FIG. 5. Mid-power photomicrograph of Ieiomyosarcoma showing typica pattern from node. The same pattern was demonstrated in tumorous tissue taken from the jcjunum. (Original mngnifrcation X 120.)
FIG. 6. Migh power photomicrograph Ieiomyosarcoma showing typical pattern in jejunum. The same pattern was demonstrated in tumorous tissue taken from nodes. (Original magnification X 480.1
later. The patient was on a reguIar diet at the time of discharge. During pathologic examination of the specimen obtained during the operation (Figs. 3 and 4), the small finger of the examiner could bareIy be passed through the partially occIuded lumen at the tumor sites. The cut section of the tumor demonstrated firm tan fIeshy tissue predominantly intramural, extending from the Iuminal surface throughout the entire wal1. The luminal surface above the tumor was ulcerated, hemorrhagic and focally covered with necrotic debris. The serosa was not penetrated by the tumor. The mesenteric lymph nodes were free of tumorous tissue. HistoIogic sections of the leiomyosarcoma of the jejunum were precisely
similar
to the
metastases
the inguinal lymph nodes. biopsy of the liver disclosed
in
previously
observed in (Figs. 5 and 6.) The normal hrpatic tissue.
COMMENTS
In this case, several interesting features were demonstrated. Since the patient had a mass in the right side of the groin at the outset without gastrointestina1 symptoms, the possibiIity of a tumor in the smaI1 bowe1 was not conskered unti1 after excision biopsy of the node. The persistent resuIts of tests for occult bIood in the stoo1 and the hemoglobin value of 863
Das Gupta,
KoIb and Gruhn SUMMARY
10.1 gm. per cent directed our attention to a bIeeding source in the gastrointestina1 tract. Intestinal bleeding has been present in 53 per cent of symptomatic patients [5]. Moreover, pathoIogic examination of the specimen revealed an impending obstruction which occurs in 70 to 76 per cent of patients. Roentgenologic examination of the upper gastrointestinal tract has provided previousIy a correct preoperative diagnosis of a tumor in the smaII bowel in 84 per cent of the patients. MuItipIicity of intestina1 sites of Ieiomyosarcoma is uncommon. OnIy two of the fortyone cases reported by Starr and Dockerty were multipIe [I]. In the instance reported herein, the Iesions appeared autochthonous rather than metastatic within the jejunum. The rare occurrence of Iymph node metastases from Ieiomyosarcoma of the smaII bowe1 shouId not be dismissed entireIy when pIanning surgica1 excision of these tumors.
A case of Ieiomyosarcomas of the jejunum of a patient with obvious Iymph node metastases in the right side of the groin is reported. This is the first reported case of Ieiomyosarcomas of the jejunum metastasizing to inguina1 lymph nodes. REFERENCES I. STARR, G. F. and DOCKERTY, M.
B. Leiomyomas and Ieiomyosarcomas of the small intestine. Cancer, 8: 101, 1955. z. HORSELY. W. G. and MEANS. L. R. Leiomvosarcoma of the jejunum, with two case reports and a review of the literature. Ann. Surg., 141: 7999 1955. 3. DARLING, R. C. and WELCH, C. E. Tumors of the smaI1 intestine. New England J. Med., 260: 397, 1959. 4. HIJMPHREYS, E. M. Personal communication, 1960. 5. WEINSTEIN, M. and ROBERTS, M. Leiomyosarcoma of the duodenum. Report of a case and summary of the literature. Arch. Surg., 66: 318, 1953.
864