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This section features outstanding photographs of clinical materials selected for their educational value or message, or possibly their rarity. The images are accompanied by brief case reports (limit 2 typed pages, 4 references). Our readers are invited to submit items for consideration.
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.
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).
Fig. 1CT scan of patient's dedifferentiated liposarcoma demonstrating liposarcoma portions (dark) with colon compressed and draped over tumor surface.
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.
Fig. 2Cut surface of tumor: Large nodules at the bottom were composed of inflammatory MFH. A portion of kidney can be seen on lower right.
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.
was histologically similar to ours, reporting a retroperitoneal liposarcoma with an inflammatory MFH dedifferentiated component in a 69-year-old man. Surgery was performed in our case and 2 of the others
The inflammatory and leukemoid reactions are directly induced by the tumor. Several cytokines (G-CSF, 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.
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.
References
Matsumoto M
Yazawa Y
Kanzaki M.
An autopsy case of liposarcoma with granulocytic leukemoid reaction.
*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.
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