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A leukemoid reaction in a patient with a dedifferentiated liposarcoma
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A leukemoid reaction in a patient with a dedifferentiated liposarcoma Selim M. Nasser, MD, Umar H. Choudry, MD, Gunnlaugur P. Nielsen, MD, and Mark J. Ott, MD, Boston, Mass
From the Department of Surgery, Division of Surgical Oncology, and the Department of Pathology, Massachusetts General Hospital, Boston, Mass
THE PATIENT WAS a 63-year-old man who in 1992 went to an outside hospital with vague abdominal discomfort. A computed tomography (CT) scan demonstrated a 4-cm mass near the tail of the pancreas. Laboratory studies revealed a white blood cell count (WBC) of 22,600/mL. The mass was excised, including a distal pancreatectomy and splenectomy. Postoperatively, the patient’s WBC normalized. Histology was read as a nonspecific inflammatory process. In 1996 he went to the outside hospital with an episode of upper gastrointestinal bleeding and ongoing melena. His laboratory findings revealed a WBC count of 33,000/mL and a hemoglobin level of 4 g/dL. Upper endoscopy demonstrated a mass eroding into the greater curvature of the stomach. After stabilization, a subtotal gastrectomy with partial excision of a retroperitoneal mass was performed. Histology demonstrated a low-grade malignant fibrous histiocytoma (MFH). The patient recovered, but his WBC remained elevated. A CT scan demonstrated a residual 14-cm retroperitoneal mass. The tumor was thought to be unresectable, and the patient was offered no further therapy. Accepted for publication May 28, 2000. Reprint requests: Mark J Ott, MD, Department of Surgery, Division of Surgical Oncology, Massachusetts General Hospital, 626 Cox Building, 100 Blossom St, Boston, MA 02114. Surgery 2001;129:765-7. Copyright © 2001 by Mosby, Inc. 0039-6060/2001/$35.00 + 0 11/60/109498 doi:10.1067/msy.2001.109498
In March 1998, the patient was referred to our institution for erosion of the tumor into the stomach with ongoing melena. A CT scan demonstrated a 48-cm mass (Fig 1). His WBC was 46,900/mL and his hemoglobin level was 8.3 g/dL. A complete resection of the retroperitoneal tumor—including the residual stomach, left colon, left kidney, and adrenal gland—was performed. Reconstruction was by means of an esophagojejunostomy and colocolostomy. The 9.5-kg tumor was mostly well-encapsulated except for the erosion into the stomach. On the cut surface, the tumor was tan-pink (soft) with 2 gray-to-yellow (firm), well-demarcated areas measuring 15 cm and 8 cm in greatest diameter (Fig 2), the latter in continuity with the mass involving the stomach. Most of the tumor consisted of a well-differentiated liposarcoma grade 1/3. The 2 gray firm areas and the gastric mass were composed of inflammatory MFH, grade 2-3/3. All margins of resection were negative. The patient made an uneventful recovery and his WBC normalized. He is doing well and is free of disease 22 months after operation with a WBC of 5600/mL. DISCUSSION We describe a dedifferentiated retroperitoneal liposarcoma in which the dedifferentiated component was an inflammatory MFH, associated clinically with a leukemoid reaction. Liposarcomas with leukemoid reactions have been rarely reported. We found only 3 cases in the literature.1-3 The first case1 was an autopsy of SURGERY 765
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Fig 1. CT scan of patient’s dedifferentiated liposarcoma demonstrating liposarcoma portions (dark) with colon compressed and draped over tumor surface.
Fig 2. Cut surface of tumor: Large nodules at the bottom were composed of inflammatory MFH. A portion of kidney can be seen on lower right.
a 57-year-old woman with a retroperitoneal pleomorphic liposarcoma. The second case2 was a 77year-old woman with a mesenteric pleomorphic liposarcoma who also had a retroperitoneal welldifferentiated liposarcoma. The third case3 was histologically similar to ours, reporting a retroperitoneal liposarcoma with an inflammatory MFH dedifferentiated component in a 69-yearold man. Surgery was performed in our case and 2 of the others 2,3 leading to resolution of the leukemoid reaction.
The inflammatory and leukemoid reactions are directly induced by the tumor. Several cytokines (GCSF, IL-6, IL-7, IL-8, IFN) have been detected in the cells of inflammatory MFH with leukemoid reaction along with elevated levels of cytokines in the sera of these patients.2-4 To date, there is no clear prognostic significance associated with leukemoid reactions or inflammatory infiltrates accompanying sarcomas. Operation should still be the primary treatment for this unique variant of liposarcoma.
Nasser et al 767
Surgery Volume 129, Number 6 REFERENCES 1. Matsumoto M, Yazawa Y, Kanzaki M. An autopsy case of liposarcoma with granulocytic leukemoid reaction. Acta Pathol Jpn 1976;26:399-408. 2. Nakamura A, Tanaka S, Takayama H, Sakamoto M, Ishii H, Kusano M, et al. Mesenteric liposarcoma with production of granulocyte colony-stimulating factor. Intern Med 1998;37:884-90.
3. Hisaoka M, Tsuji S, Hashimoto H, Aoki T, Uriu K. Dedifferentiated liposarcoma with an inflammatory malignant fibrous histiocytoma-like component presenting a leukemoid reaction. Pathol Int 1997;47:642-6. 4. Melhem M, Meisler A, Saito R, Finley G, Hockman H, Koski R. Cytokines in inflammatory malignant fibrous histiocytoma presenting with a leukemoid reaction. Blood 1993;82:2038-44.
Acknowledgment We would like to thank the reviewers listed below who contributed their time recently to review manuscripts for Surgery. These individuals, as well as members of the Editorial Board, commit their time and careful consideration to ensure that articles in Surgery reflect the highest standards of scholarship and relevance. Andrew L. Warshaw Michael G. Sarr Editors in Chief Atomi, Yutaka Kyorin University School of Medicine Barksdale, Jr, Edward M. Children’s Hospital of Pittsburgh Bolton, John S. Ochsner Clinic Brunicardi, F. Charles Methodist Hospital/Baylor College of Medicine Choti, Michael A. The Johns Hopkins Hospital Conlon, Kevin C. P. Memorial Sloan-Kettering Cancer Center Dawes, Lillian G. Ann Arbor VAMC Doherty, Gerard M. Washington University School of Medicine Donahue, Philip E. Cook County Hospital Doody, Daniel P. Massachusetts General Hospital Duh, Quan-Yang VA Medical Center San Francisco Economou, James S. UCLA Medical Center Fernandez-del Castillo, Carlos Massachusetts General Hospital Fleshman, James Washington University School of Medicine Fry, Robert D. Thomas Jefferson University Galandiak, Susan University of Louisville Garrison, Neal R. University of Louisville Greenfield, Lazar University of Michigan Hewett, Peter J. The Queen Elizabeth Hospital/University of Adelaide Hoffman, William Massachusetts General Hospital Kathouda, Namir USC Healthcare Consultation Center Kendrick, Michael Mayo Clinic Klar, Ernest Klinikum der Universitat Heidelberg Lam, Chi-Ming The University of Hong Kong/Queen Mary Hospital
Libsch, Karen Mayo Clinic Lilemoe, Keith D. Johns Hopkins Hospital Meyers, William C. MCP Hahnemann University Michelassi, Fabrizio University of Chicago Miyazaki, Kohji Saga Medical School Moley, Jeffrey F. Washington University School of Medicine Nagoney, David M. Mayo Clinic Nelson, Heidi Mayo Clinic Norton, Jeffrey A. VA Medical Center San Francisco Prinz, Richard A. Rush-Presbyterian–St. Luke’s Medical Rege, Robert V. University of Texas/Southwestern Medical Center Reilly, Patrick The Trauma Center at Penn Rikkers, Layton University of Wisconsin Clinical Science Center Sitzmann, James V. University of Rochester Soper, Nathaniel J. Washington University School of Medicine Steele, Jr, Glenn D. University of Chicago Tam, Paul The University of Hong Kong/Queen Mary Hospital Tanabe, Kenneth K. Massachusetts General Hospital Thayer, Sarah Massachusetts General Hospital Thompson, Norman W. University of Michigan Medical Center Tsiotos, Greg Greece Udelsman, Robert The Johns Hopkins Hospital Wong, John The University of Hong Kong/Queen Mary Hospital Young-Fadok, Tonia M. Mayo Clinic
A leukemoid reaction in a patient with a dedifferentiated liposarcoma Selim M. Nasser, MD, Umar H. Choudry, MD, Gunnlaugur P. Nielsen, MD, and Mark J. Ott, MD, Boston, Mass
From the Department of Surgery, Division of Surgical Oncology, and the Department of Pathology, Massachusetts General Hospital, Boston, Mass
THE PATIENT WAS a 63-year-old man who in 1992 went to an outside hospital with vague abdominal discomfort. A computed tomography (CT) scan demonstrated a 4-cm mass near the tail of the pancreas. Laboratory studies revealed a white blood cell count (WBC) of 22,600/mL. The mass was excised, including a distal pancreatectomy and splenectomy. Postoperatively, the patient’s WBC normalized. Histology was read as a nonspecific inflammatory process. In 1996 he went to the outside hospital with an episode of upper gastrointestinal bleeding and ongoing melena. His laboratory findings revealed a WBC count of 33,000/mL and a hemoglobin level of 4 g/dL. Upper endoscopy demonstrated a mass eroding into the greater curvature of the stomach. After stabilization, a subtotal gastrectomy with partial excision of a retroperitoneal mass was performed. Histology demonstrated a low-grade malignant fibrous histiocytoma (MFH). The patient recovered, but his WBC remained elevated. A CT scan demonstrated a residual 14-cm retroperitoneal mass. The tumor was thought to be unresectable, and the patient was offered no further therapy. Accepted for publication May 28, 2000. Reprint requests: Mark J Ott, MD, Department of Surgery, Division of Surgical Oncology, Massachusetts General Hospital, 626 Cox Building, 100 Blossom St, Boston, MA 02114. Surgery 2001;129:765-7. Copyright © 2001 by Mosby, Inc. 0039-6060/2001/$35.00 + 0 11/60/109498 doi:10.1067/msy.2001.109498
In March 1998, the patient was referred to our institution for erosion of the tumor into the stomach with ongoing melena. A CT scan demonstrated a 48-cm mass (Fig 1). His WBC was 46,900/mL and his hemoglobin level was 8.3 g/dL. A complete resection of the retroperitoneal tumor—including the residual stomach, left colon, left kidney, and adrenal gland—was performed. Reconstruction was by means of an esophagojejunostomy and colocolostomy. The 9.5-kg tumor was mostly well-encapsulated except for the erosion into the stomach. On the cut surface, the tumor was tan-pink (soft) with 2 gray-to-yellow (firm), well-demarcated areas measuring 15 cm and 8 cm in greatest diameter (Fig 2), the latter in continuity with the mass involving the stomach. Most of the tumor consisted of a well-differentiated liposarcoma grade 1/3. The 2 gray firm areas and the gastric mass were composed of inflammatory MFH, grade 2-3/3. All margins of resection were negative. The patient made an uneventful recovery and his WBC normalized. He is doing well and is free of disease 22 months after operation with a WBC of 5600/mL. DISCUSSION We describe a dedifferentiated retroperitoneal liposarcoma in which the dedifferentiated component was an inflammatory MFH, associated clinically with a leukemoid reaction. Liposarcomas with leukemoid reactions have been rarely reported. We found only 3 cases in the literature.1-3 The first case1 was an autopsy of SURGERY 765
766 Nasser et al
Surgery June 2001
Fig 1. CT scan of patient’s dedifferentiated liposarcoma demonstrating liposarcoma portions (dark) with colon compressed and draped over tumor surface.
Fig 2. Cut surface of tumor: Large nodules at the bottom were composed of inflammatory MFH. A portion of kidney can be seen on lower right.
a 57-year-old woman with a retroperitoneal pleomorphic liposarcoma. The second case2 was a 77year-old woman with a mesenteric pleomorphic liposarcoma who also had a retroperitoneal welldifferentiated liposarcoma. The third case3 was histologically similar to ours, reporting a retroperitoneal liposarcoma with an inflammatory MFH dedifferentiated component in a 69-yearold man. Surgery was performed in our case and 2 of the others 2,3 leading to resolution of the leukemoid reaction.
The inflammatory and leukemoid reactions are directly induced by the tumor. Several cytokines (GCSF, IL-6, IL-7, IL-8, IFN) have been detected in the cells of inflammatory MFH with leukemoid reaction along with elevated levels of cytokines in the sera of these patients.2-4 To date, there is no clear prognostic significance associated with leukemoid reactions or inflammatory infiltrates accompanying sarcomas. Operation should still be the primary treatment for this unique variant of liposarcoma.
Nasser et al 767
Surgery Volume 129, Number 6 REFERENCES 1. Matsumoto M, Yazawa Y, Kanzaki M. An autopsy case of liposarcoma with granulocytic leukemoid reaction. Acta Pathol Jpn 1976;26:399-408. 2. Nakamura A, Tanaka S, Takayama H, Sakamoto M, Ishii H, Kusano M, et al. Mesenteric liposarcoma with production of granulocyte colony-stimulating factor. Intern Med 1998;37:884-90.
3. Hisaoka M, Tsuji S, Hashimoto H, Aoki T, Uriu K. Dedifferentiated liposarcoma with an inflammatory malignant fibrous histiocytoma-like component presenting a leukemoid reaction. Pathol Int 1997;47:642-6. 4. Melhem M, Meisler A, Saito R, Finley G, Hockman H, Koski R. Cytokines in inflammatory malignant fibrous histiocytoma presenting with a leukemoid reaction. Blood 1993;82:2038-44.
Acknowledgment We would like to thank the reviewers listed below who contributed their time recently to review manuscripts for Surgery. These individuals, as well as members of the Editorial Board, commit their time and careful consideration to ensure that articles in Surgery reflect the highest standards of scholarship and relevance. Andrew L. Warshaw Michael G. Sarr Editors in Chief Atomi, Yutaka Kyorin University School of Medicine Barksdale, Jr, Edward M. Children’s Hospital of Pittsburgh Bolton, John S. Ochsner Clinic Brunicardi, F. Charles Methodist Hospital/Baylor College of Medicine Choti, Michael A. The Johns Hopkins Hospital Conlon, Kevin C. P. Memorial Sloan-Kettering Cancer Center Dawes, Lillian G. Ann Arbor VAMC Doherty, Gerard M. Washington University School of Medicine Donahue, Philip E. Cook County Hospital Doody, Daniel P. Massachusetts General Hospital Duh, Quan-Yang VA Medical Center San Francisco Economou, James S. UCLA Medical Center Fernandez-del Castillo, Carlos Massachusetts General Hospital Fleshman, James Washington University School of Medicine Fry, Robert D. Thomas Jefferson University Galandiak, Susan University of Louisville Garrison, Neal R. University of Louisville Greenfield, Lazar University of Michigan Hewett, Peter J. The Queen Elizabeth Hospital/University of Adelaide Hoffman, William Massachusetts General Hospital Kathouda, Namir USC Healthcare Consultation Center Kendrick, Michael Mayo Clinic Klar, Ernest Klinikum der Universitat Heidelberg Lam, Chi-Ming The University of Hong Kong/Queen Mary Hospital
Libsch, Karen Mayo Clinic Lilemoe, Keith D. Johns Hopkins Hospital Meyers, William C. MCP Hahnemann University Michelassi, Fabrizio University of Chicago Miyazaki, Kohji Saga Medical School Moley, Jeffrey F. Washington University School of Medicine Nagoney, David M. Mayo Clinic Nelson, Heidi Mayo Clinic Norton, Jeffrey A. VA Medical Center San Francisco Prinz, Richard A. Rush-Presbyterian–St. Luke’s Medical Rege, Robert V. University of Texas/Southwestern Medical Center Reilly, Patrick The Trauma Center at Penn Rikkers, Layton University of Wisconsin Clinical Science Center Sitzmann, James V. University of Rochester Soper, Nathaniel J. Washington University School of Medicine Steele, Jr, Glenn D. University of Chicago Tam, Paul The University of Hong Kong/Queen Mary Hospital Tanabe, Kenneth K. Massachusetts General Hospital Thayer, Sarah Massachusetts General Hospital Thompson, Norman W. University of Michigan Medical Center Tsiotos, Greg Greece Udelsman, Robert The Johns Hopkins Hospital Wong, John The University of Hong Kong/Queen Mary Hospital Young-Fadok, Tonia M. Mayo Clinic