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Leiomyosarcoma Originating from the Superior Mesenteric Vein: A Case Report and Review of the Literature
Leiomyosarcoma Originating from the Superior Mesenteric Vein: A Case Report and Review of the Literature Sosei Kuma,1 Tohru Utsunomiya,1 Shuya Yano,1 Toshifumi Kameyama,1 Masahiro Okamoto,1 Ayumi Matsuyama,1 Kohji Hashimoto,1 Manabu Yamamoto,1 Takahiro Ezaki,2 Megumu Fujihara,3 and Teruyoshi Ishida,1 Hiroshima and Kyushu, Japan
We describe a rare case of leiomyosarcoma originating from the superior mesenteric vein with concomitant liver metastases in a 62-year-old woman. She underwent a tumor resection and venous reconstruction, right hemicolectomy, and right hepatic lobectomy. Since the tumor was weakly positive for c-kit, she was treated with imatinib mesylate for the recurrent liver tumors. She has survived for about 3 years after undergoing the surgical procedures.
Vascular leiomyosarcomas are rare. They arise predominantly from larger and medium-sized blood vessels, the inferior vena cava accounting for over 75% of these cases.1,2 Leiomyosarcomas arising from the portal venous system are extremely rare. There have only been four cases reported in the English literature to our knowledge.3-6 We herein report an advanced-staged case of leiomyosarcoma of the superior mesenteric vein (SMV), which was surgically resected, with the mesenteric venous flow being reconstructed.
CASE REPORT A 62-year-old female was found to have a right upper abdominal mass by her masseur and, thus, admitted to our hospital. She had no complaints such as abdominal pain, nausea, or appetite loss until admission. The physical examination showed a nontender abdominal mass in her right upper quadrant. The peripheral blood count and 1 Department of Surgery, Hiroshima Red Cross Hospital and Atomic Bomb Survivors’ Hospital, Hiroshima 730-8619, Japan. 2 Department of Surgery, Fukuoka Higashi Medical Center, Koga 811-3195, Japan. 3 Department of Pathology, Hiroshima Red Cross Hospital and Atomic Bomb Survivors’ Hospital, Hiroshima 730-8619, Japan. Correspondence to: Sosei Kuma, MD, E-mail: kumasosei@yahoo. co.jp
Ann Vasc Surg 2008; 22: 453-455 DOI: 10.1016/j.avsg.2007.12.008 Ó Annals of Vascular Surgery Inc. Published online: March 24, 2008
biochemical test results were within the normal range, except for a slight elevation of the lactate dehydrogenase level. Abdominal computed tomography (CT) demonstrated a well-defined heterogeneously enhanced, lobulated tumor in the retroperitoneum, which measured 13 10 7 cm, and two liver metastatic nodules, measuring 4.5 and 1.0 cm, in the right hepatic lobe. The tumor was noted to have defined areas of hypoattenuation, which were thought to represent cystic or necrotic changes. Magnetic resonance imaging (Fig. 1) and portography revealed severe stenosis of the SMV. A laparotomy confirmed a lobulated tumor arising from the anterior wall of the SMV and three hepatic nodules in the right hepatic lobe. The tumor also grew in the right colonic mesentery along the medial colic vessels. There was no evidence of metastasis to the regional lymph nodes or peritoneal dissemination. We determined the tumor to have originated from the SMV because it was mobilized from all surrounding tissue and clearly attached to the anterior wall of the SMV. The segment of SMV containing the tumor was isolated by dividing the middle colic vessels, and then the tumor was removed. The mesenteric venous flow was restored through end-to-end anastomosis. Because the tumor was located just inferior to the end of the pancreas, the surgical margin excised around the tumor was relatively short. A pancreatoduodenectomy might be required to sufficiently secure the surgical margin, but the patient might not tolerate such an extensive procedure. Consequently, we mobilized the proximal and distal ends of the transected vein and reconstructed with a direct reconnection. Neither systemic heparinization nor a temporary shunt was performed. A right hepatic lobectomy was performed. A right hemicolectomy was also performed for colonic ischemia, which was recognized after dividing the middle colic vessels. The perioperative course was 453
454 Case reports
Annals of Vascular Surgery
Fig. 1. Magnetic resonance images showing a well-defined tumor, which appeared to originate from the SMV. Left: coronac image, Right: axial image.
Fig. 2. Microscopic findings (hematoxylin & eosin stain). a The tumor was rich in cellularity and consisted of spindle-shaped cells with eosinophilic cytoplasm (100).
b High-power magnification (400) revealed nuclear pleomorphism and cellular atypia.
uneventful. The cut surface of the tumor showed homogeneous firm white tissue, including some degeneration and necrosis. Microscopically, the tumor was made up of interlacing bundles of neoplastic spindle cells infiltrating the wall of the mesenteric vein without involving the surgical margins. The nuclei were elongated, with hyperchromasia and variation in size and shape; and 115 mitoses were counted in 10 high-power fields (Fig. 2). Special staining was positive for a-smooth muscle actin, desmin, and epithelium membrane antigen and weakly positive for c-kit. At the 8-month follow-up, multiple metastatic nodules in the left hepatic lobe were detected by abdominal CT. Gain-of-function mutations of the c-kit gene have been observed in gastrointestinal stromal tumors (GISTs), and a selective inhibitor of tyrosine kinase activity of the c-kit, imatinib mesylate, has been reported to be an effective therapy for GIST. The patient was therefore administered imatinib mesylate at 13 months after
the operation. Despite receiving such treatments, the follow-up CT scans showed lymph node and lung metastases in addition to gradual growth of the liver metastasis. At 33 months after surgery, she demonstrated obstructive jaundice due to growth of the lymph nodes in the hepatoduodenal ligament.
DISCUSSION Leiomyosarcomas originating from smooth muscle are the most common type of soft tissue sarcoma. The majority of leiomyosarcomas tend to be visceral in origin, arising from the smooth muscle of the intestine or uterus; and vascular leiomyosarcomas are rare.1 Leiomyosarcomas arising from the portal venous system are extremely rare, and to the best of
Vol. 22, No. 3, 2008
Case reports 455
Table I. Clinical characteristics of the previous four cases and our case of primary leiomyosarcoma arising from the portal venous system Age (years)/ sex
Tumor size (cm)
Location
Operation
Additional therapy
Outcome
Reference
28/F 28/F
Unknown 4.7
PV PV
Exploration Mesocaval shunt, TR & VR TR & VR
None Radiation (45 Gy)
Unknown Alive (6 years)
3 4
Radiation (50 Gy), hepatectomy None
Alive (12 months)
5
Alive (6 months)
6
Imatinib mesylate
Alive (33 months)
Our case
40/F
8
SMV
60/M
4
SMV
62/F
13
SMV
TR & VR, hemicolectomy TR & VR, hemicolectomy hepatectomy
PV, portal vein; SMV, superior mesenteric vein; TR & VR, tumor resection and venous reconstruction.
our knowledge, only four such cases3-6 have been previously reported in the English literature. These four previous cases and our case are all summarized in Table I. These include four women and one man of median age 44 years (range 28-62). Their complaints included appetite loss, upper abdominal discomfort/pain, and abdominal fullness. The tumors of two cases originated from the portal vein, while those of the other three originated from the SMV. The median size was 7.5 cm (range 4-13), and our case was the largest in size of all five cases. The first choice of treatment for venous leiomyosarcoma is a complete radical excision, usually with venous reconstruction.2 Two cases of portal vein leiomyosarcoma had portal vein obstruction with extensive collaterals. As a result, one tumor was unresectable3 and the other was resected 6 months after constructing a mesocaval shunt.4 In contrast, both a tumor resection and venous reconstruction were successfully performed in the three cases with SMV leiomyosarcoma. Two of these cases additionally underwent a right hemicolectomy because of colonic ischemia. The tumor resectability and type of operation may depend upon the location of the tumor in the portal venous system. Even after a complete resection, the patients remain at high risk for recurrence. Radiation therapy regimens are often used for local control, although there is no definite evidence of any clinical benefit. Various chemotherapy regimens, such as doxorubicin, vincristine, dimethyl triazeno imidazole carboxamide, cyclophosphamide, methotrexate, and ifosfamide, have been tried for
venous leiomyosarcomas, although no extensive clinical studies using these regimens have been documented. Our patient was treated with imatinib mesylate because the tumor was weakly positive for c-kit, but it was not found to have any substantial impact on the treatment. Leiomyosarcoma is a malignant tumor, and a good prognosis can be expected if the tumor is extirpated. In the present case, we simultaneously performed a tumor resection and venous reconstruction, right hemicolectomy, and right hepatic lobectomy. In view of the prolonged survival of this patient of about 3 years, such an approach appears to be a reasonable and effective management strategy for patients with advanced-stage leiomyosarcomas originating from the portal venous system.
REFERENCES 1. Kevorkian J, Cento DP. Leiomyosarcoma of large arteries and veins. Surgery 1973;73:390-400. 2. Mingoli A, Cavallaro A, Sapienza P, et al. International registry of inferior vena cava leiomyosarcoma: analysis of world series on 218 patients. Anticancer Res 1996;16:32013206. 3. Wilson SR, Hine AL. Leiomyosarcoma of the portal vein. AJR Am J Roentgenol 1987;149:183-184. 4. Madariaga JR, Nalesnik MA, Iwatsuki S, et al. Spindle cell tumor of the portal vein. J Surg Oncol 1995;59:139-141. 5. Goldin SB, Webb TH, Lillemoe KD. Leiomyosarcoma arising from the superior mesenteric vein. Surgery 2002;132: 108-109. 6. Celdran A, Frieyro O, del Rio A, et al. Leiomyosarcoma of the portal venous system: a case report and review of literature. Surgery 2004;135:455-456.
We describe a rare case of leiomyosarcoma originating from the superior mesenteric vein with concomitant liver metastases in a 62-year-old woman. She underwent a tumor resection and venous reconstruction, right hemicolectomy, and right hepatic lobectomy. Since the tumor was weakly positive for c-kit, she was treated with imatinib mesylate for the recurrent liver tumors. She has survived for about 3 years after undergoing the surgical procedures.
Vascular leiomyosarcomas are rare. They arise predominantly from larger and medium-sized blood vessels, the inferior vena cava accounting for over 75% of these cases.1,2 Leiomyosarcomas arising from the portal venous system are extremely rare. There have only been four cases reported in the English literature to our knowledge.3-6 We herein report an advanced-staged case of leiomyosarcoma of the superior mesenteric vein (SMV), which was surgically resected, with the mesenteric venous flow being reconstructed.
CASE REPORT A 62-year-old female was found to have a right upper abdominal mass by her masseur and, thus, admitted to our hospital. She had no complaints such as abdominal pain, nausea, or appetite loss until admission. The physical examination showed a nontender abdominal mass in her right upper quadrant. The peripheral blood count and 1 Department of Surgery, Hiroshima Red Cross Hospital and Atomic Bomb Survivors’ Hospital, Hiroshima 730-8619, Japan. 2 Department of Surgery, Fukuoka Higashi Medical Center, Koga 811-3195, Japan. 3 Department of Pathology, Hiroshima Red Cross Hospital and Atomic Bomb Survivors’ Hospital, Hiroshima 730-8619, Japan. Correspondence to: Sosei Kuma, MD, E-mail: kumasosei@yahoo. co.jp
Ann Vasc Surg 2008; 22: 453-455 DOI: 10.1016/j.avsg.2007.12.008 Ó Annals of Vascular Surgery Inc. Published online: March 24, 2008
biochemical test results were within the normal range, except for a slight elevation of the lactate dehydrogenase level. Abdominal computed tomography (CT) demonstrated a well-defined heterogeneously enhanced, lobulated tumor in the retroperitoneum, which measured 13 10 7 cm, and two liver metastatic nodules, measuring 4.5 and 1.0 cm, in the right hepatic lobe. The tumor was noted to have defined areas of hypoattenuation, which were thought to represent cystic or necrotic changes. Magnetic resonance imaging (Fig. 1) and portography revealed severe stenosis of the SMV. A laparotomy confirmed a lobulated tumor arising from the anterior wall of the SMV and three hepatic nodules in the right hepatic lobe. The tumor also grew in the right colonic mesentery along the medial colic vessels. There was no evidence of metastasis to the regional lymph nodes or peritoneal dissemination. We determined the tumor to have originated from the SMV because it was mobilized from all surrounding tissue and clearly attached to the anterior wall of the SMV. The segment of SMV containing the tumor was isolated by dividing the middle colic vessels, and then the tumor was removed. The mesenteric venous flow was restored through end-to-end anastomosis. Because the tumor was located just inferior to the end of the pancreas, the surgical margin excised around the tumor was relatively short. A pancreatoduodenectomy might be required to sufficiently secure the surgical margin, but the patient might not tolerate such an extensive procedure. Consequently, we mobilized the proximal and distal ends of the transected vein and reconstructed with a direct reconnection. Neither systemic heparinization nor a temporary shunt was performed. A right hepatic lobectomy was performed. A right hemicolectomy was also performed for colonic ischemia, which was recognized after dividing the middle colic vessels. The perioperative course was 453
454 Case reports
Annals of Vascular Surgery
Fig. 1. Magnetic resonance images showing a well-defined tumor, which appeared to originate from the SMV. Left: coronac image, Right: axial image.
Fig. 2. Microscopic findings (hematoxylin & eosin stain). a The tumor was rich in cellularity and consisted of spindle-shaped cells with eosinophilic cytoplasm (100).
b High-power magnification (400) revealed nuclear pleomorphism and cellular atypia.
uneventful. The cut surface of the tumor showed homogeneous firm white tissue, including some degeneration and necrosis. Microscopically, the tumor was made up of interlacing bundles of neoplastic spindle cells infiltrating the wall of the mesenteric vein without involving the surgical margins. The nuclei were elongated, with hyperchromasia and variation in size and shape; and 115 mitoses were counted in 10 high-power fields (Fig. 2). Special staining was positive for a-smooth muscle actin, desmin, and epithelium membrane antigen and weakly positive for c-kit. At the 8-month follow-up, multiple metastatic nodules in the left hepatic lobe were detected by abdominal CT. Gain-of-function mutations of the c-kit gene have been observed in gastrointestinal stromal tumors (GISTs), and a selective inhibitor of tyrosine kinase activity of the c-kit, imatinib mesylate, has been reported to be an effective therapy for GIST. The patient was therefore administered imatinib mesylate at 13 months after
the operation. Despite receiving such treatments, the follow-up CT scans showed lymph node and lung metastases in addition to gradual growth of the liver metastasis. At 33 months after surgery, she demonstrated obstructive jaundice due to growth of the lymph nodes in the hepatoduodenal ligament.
DISCUSSION Leiomyosarcomas originating from smooth muscle are the most common type of soft tissue sarcoma. The majority of leiomyosarcomas tend to be visceral in origin, arising from the smooth muscle of the intestine or uterus; and vascular leiomyosarcomas are rare.1 Leiomyosarcomas arising from the portal venous system are extremely rare, and to the best of
Vol. 22, No. 3, 2008
Case reports 455
Table I. Clinical characteristics of the previous four cases and our case of primary leiomyosarcoma arising from the portal venous system Age (years)/ sex
Tumor size (cm)
Location
Operation
Additional therapy
Outcome
Reference
28/F 28/F
Unknown 4.7
PV PV
Exploration Mesocaval shunt, TR & VR TR & VR
None Radiation (45 Gy)
Unknown Alive (6 years)
3 4
Radiation (50 Gy), hepatectomy None
Alive (12 months)
5
Alive (6 months)
6
Imatinib mesylate
Alive (33 months)
Our case
40/F
8
SMV
60/M
4
SMV
62/F
13
SMV
TR & VR, hemicolectomy TR & VR, hemicolectomy hepatectomy
PV, portal vein; SMV, superior mesenteric vein; TR & VR, tumor resection and venous reconstruction.
our knowledge, only four such cases3-6 have been previously reported in the English literature. These four previous cases and our case are all summarized in Table I. These include four women and one man of median age 44 years (range 28-62). Their complaints included appetite loss, upper abdominal discomfort/pain, and abdominal fullness. The tumors of two cases originated from the portal vein, while those of the other three originated from the SMV. The median size was 7.5 cm (range 4-13), and our case was the largest in size of all five cases. The first choice of treatment for venous leiomyosarcoma is a complete radical excision, usually with venous reconstruction.2 Two cases of portal vein leiomyosarcoma had portal vein obstruction with extensive collaterals. As a result, one tumor was unresectable3 and the other was resected 6 months after constructing a mesocaval shunt.4 In contrast, both a tumor resection and venous reconstruction were successfully performed in the three cases with SMV leiomyosarcoma. Two of these cases additionally underwent a right hemicolectomy because of colonic ischemia. The tumor resectability and type of operation may depend upon the location of the tumor in the portal venous system. Even after a complete resection, the patients remain at high risk for recurrence. Radiation therapy regimens are often used for local control, although there is no definite evidence of any clinical benefit. Various chemotherapy regimens, such as doxorubicin, vincristine, dimethyl triazeno imidazole carboxamide, cyclophosphamide, methotrexate, and ifosfamide, have been tried for
venous leiomyosarcomas, although no extensive clinical studies using these regimens have been documented. Our patient was treated with imatinib mesylate because the tumor was weakly positive for c-kit, but it was not found to have any substantial impact on the treatment. Leiomyosarcoma is a malignant tumor, and a good prognosis can be expected if the tumor is extirpated. In the present case, we simultaneously performed a tumor resection and venous reconstruction, right hemicolectomy, and right hepatic lobectomy. In view of the prolonged survival of this patient of about 3 years, such an approach appears to be a reasonable and effective management strategy for patients with advanced-stage leiomyosarcomas originating from the portal venous system.
REFERENCES 1. Kevorkian J, Cento DP. Leiomyosarcoma of large arteries and veins. Surgery 1973;73:390-400. 2. Mingoli A, Cavallaro A, Sapienza P, et al. International registry of inferior vena cava leiomyosarcoma: analysis of world series on 218 patients. Anticancer Res 1996;16:32013206. 3. Wilson SR, Hine AL. Leiomyosarcoma of the portal vein. AJR Am J Roentgenol 1987;149:183-184. 4. Madariaga JR, Nalesnik MA, Iwatsuki S, et al. Spindle cell tumor of the portal vein. J Surg Oncol 1995;59:139-141. 5. Goldin SB, Webb TH, Lillemoe KD. Leiomyosarcoma arising from the superior mesenteric vein. Surgery 2002;132: 108-109. 6. Celdran A, Frieyro O, del Rio A, et al. Leiomyosarcoma of the portal venous system: a case report and review of literature. Surgery 2004;135:455-456.