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Malignant fibrous xanthoma of retroperitoneum
MALIGNANT
FIBROUS
XANTHOMA
OF
RETROPERITONEUM PETER LOWELL
S. ALBERT, H. KANE,
M.D. M.D.
From the Department of Urology, New York Medical College, Flower New York, New York
and Fifth Avenue
ABSTRACT-Fibrous xanthomas of the retroperitoneum able diagnostic procedure is tissue-culture technique these tumors can be confirmed.
This is a report of a malignant fibrous xanthoma of the retroperitoneum, a rare tumor entity. Case
are rare. A case is reported. The most valuwhere the neoplastic histiocytic origins of
returned with massive ascites and evidence of skin metastases. A rapid downhill course ensued despite chemotherapy.
Report
A sixty-nine-year-old white man entered the hospital with a three-month history of anorexia and a 25-pound weight loss. There was no history of nausea, vomiting, dysphagia, flank pain, dysuria, or hematuria. Pertinent physical findings were confined to the abdomen where a large mass in the upper left quadrant was palpated. It was firm, slightly movable, irregular, somewhat nodular, and occupied most of the left side of the patient’s abdomen. There was no movement of the mass on respiration. Admission laboratory data revealed hemaglobin was 10.7 Gm., hematocrit 34, erythrocyte sedimentation rate 34 per hour, a normal white cell count and differential, with normal urinalysis result. Intravenous pyelogram revealed a wellcircumscribed mass displacing the left calyceal system superiorly with no evidence of invasion into or destruction of the collecting system. Gastrointestinal series documented a displacement of the colon by the abdominal mass. With the presumptive diagnosis of lymphoma, exploratory laparotomy was performed, and a firm, large mass adherent to Gerota’s fascia and a second, smaller mass adherent to the sigmoid colon were found. Radical nephrectomy was performed with resection of the involved sigmoid colon. The patient’s postoperative course was uneventful until three months later when he
460
Hospitals,
Pathologic
diagnosis
Surrounding the kidney was a brown ovoid tumor with cystic areas filled with necrotic debris. The mass completely encompassed the kidney. The section of resected intestine revealed a metastatic lesion. Microscopic sections disclosed the renal cortex separated by a fibrous band from a highly cellular, well-defined lesion. There was no invasion into the renal parenchyma. The tumor had a sarcomatous storiform pattern with numerous giant histiocytic cells (Fig. 1). Gomori’s reticulin stain revealed fine scattered reticulin fibrils in no orderly pattern. The number of mitotic figures was approximately 30 in 50 high-power field. Final diagnosis was malignant fibrous xanthoma.* Comment Fibrous xanthomas are a rare type of tumor of the retroperitoneum. It is estimated that only 1 per cent of fibrous xanthomas prove to be malignant.’ The fibrous xanthoma has many nonmalignant counterparts. Some are associated with metabolic disturbances, such as xanthoma diabeticorum with diabetes; multiple xanthomatous giant-cell tumors of the tendon sheaths
*Confirmed by A. P. Stout, M.D., and R. L&es, Columbia University, New York, New York.
UROLOGY
/ MAY
1973
/ VOLUMELNUMBER5
M.D.,
Microscopic sections showing (A) tumor mass impinging on renal cortex with no microscopic invasion of kidney parenchyma, (B) sarcomatous storiform pattern with scattered lymphocytic infiltrate, and (C) giant histiocyte with abundant cytoplasm readily apparent throughout tumor.
FIGURE 1.
in hypercholesterolemia, and xanthomata of the eyelids seen in elderly people.2 Malignant fibrous xanthomas are classified as such by their high mitotic rate, active infiltrative growth, known metastases, and domination of the tumor by histiocytes. The diagnosis of malignant fibrous xanthoma is made by history, clinical course of the disease, special staining electron microscopy, and tissuetechniques, culture techniques. Special training and tissue cultures are most useful in differentiating sarcomatous lesions. Electron microscopy is of limited value except for the ability to demonstrate the increased protein synthesis detectable in a tumor of fibroblastic origin.
C~ROLCK:Y / MAY 1973 / VOLUME
I,NUMBER5
The diagnostic procedure of greatest value is the tissue-culture technique where the neoplastic histiocytic origins of these tumors can be confirmed. It is most important to classify these sarcomas histologically because the prognosis differs widely. Fifth Avenue New
York,
and 106th Street New
York
10029
Heferences 1. O'BRIEN, J, E., and STOUT, A. P.: hIalignant fibrous xanthomas, Cancer 17: I445 (1964). 2. STOUT, A. P.,and LATTES, R.: Tllmorsof Soft Tissues, Atlas of Tumor Pathology, Washington, D.C., Armed Forces Institute of Pathology, 1966,p.38.
461
FIBROUS
XANTHOMA
OF
RETROPERITONEUM PETER LOWELL
S. ALBERT, H. KANE,
M.D. M.D.
From the Department of Urology, New York Medical College, Flower New York, New York
and Fifth Avenue
ABSTRACT-Fibrous xanthomas of the retroperitoneum able diagnostic procedure is tissue-culture technique these tumors can be confirmed.
This is a report of a malignant fibrous xanthoma of the retroperitoneum, a rare tumor entity. Case
are rare. A case is reported. The most valuwhere the neoplastic histiocytic origins of
returned with massive ascites and evidence of skin metastases. A rapid downhill course ensued despite chemotherapy.
Report
A sixty-nine-year-old white man entered the hospital with a three-month history of anorexia and a 25-pound weight loss. There was no history of nausea, vomiting, dysphagia, flank pain, dysuria, or hematuria. Pertinent physical findings were confined to the abdomen where a large mass in the upper left quadrant was palpated. It was firm, slightly movable, irregular, somewhat nodular, and occupied most of the left side of the patient’s abdomen. There was no movement of the mass on respiration. Admission laboratory data revealed hemaglobin was 10.7 Gm., hematocrit 34, erythrocyte sedimentation rate 34 per hour, a normal white cell count and differential, with normal urinalysis result. Intravenous pyelogram revealed a wellcircumscribed mass displacing the left calyceal system superiorly with no evidence of invasion into or destruction of the collecting system. Gastrointestinal series documented a displacement of the colon by the abdominal mass. With the presumptive diagnosis of lymphoma, exploratory laparotomy was performed, and a firm, large mass adherent to Gerota’s fascia and a second, smaller mass adherent to the sigmoid colon were found. Radical nephrectomy was performed with resection of the involved sigmoid colon. The patient’s postoperative course was uneventful until three months later when he
460
Hospitals,
Pathologic
diagnosis
Surrounding the kidney was a brown ovoid tumor with cystic areas filled with necrotic debris. The mass completely encompassed the kidney. The section of resected intestine revealed a metastatic lesion. Microscopic sections disclosed the renal cortex separated by a fibrous band from a highly cellular, well-defined lesion. There was no invasion into the renal parenchyma. The tumor had a sarcomatous storiform pattern with numerous giant histiocytic cells (Fig. 1). Gomori’s reticulin stain revealed fine scattered reticulin fibrils in no orderly pattern. The number of mitotic figures was approximately 30 in 50 high-power field. Final diagnosis was malignant fibrous xanthoma.* Comment Fibrous xanthomas are a rare type of tumor of the retroperitoneum. It is estimated that only 1 per cent of fibrous xanthomas prove to be malignant.’ The fibrous xanthoma has many nonmalignant counterparts. Some are associated with metabolic disturbances, such as xanthoma diabeticorum with diabetes; multiple xanthomatous giant-cell tumors of the tendon sheaths
*Confirmed by A. P. Stout, M.D., and R. L&es, Columbia University, New York, New York.
UROLOGY
/ MAY
1973
/ VOLUMELNUMBER5
M.D.,
Microscopic sections showing (A) tumor mass impinging on renal cortex with no microscopic invasion of kidney parenchyma, (B) sarcomatous storiform pattern with scattered lymphocytic infiltrate, and (C) giant histiocyte with abundant cytoplasm readily apparent throughout tumor.
FIGURE 1.
in hypercholesterolemia, and xanthomata of the eyelids seen in elderly people.2 Malignant fibrous xanthomas are classified as such by their high mitotic rate, active infiltrative growth, known metastases, and domination of the tumor by histiocytes. The diagnosis of malignant fibrous xanthoma is made by history, clinical course of the disease, special staining electron microscopy, and tissuetechniques, culture techniques. Special training and tissue cultures are most useful in differentiating sarcomatous lesions. Electron microscopy is of limited value except for the ability to demonstrate the increased protein synthesis detectable in a tumor of fibroblastic origin.
C~ROLCK:Y / MAY 1973 / VOLUME
I,NUMBER5
The diagnostic procedure of greatest value is the tissue-culture technique where the neoplastic histiocytic origins of these tumors can be confirmed. It is most important to classify these sarcomas histologically because the prognosis differs widely. Fifth Avenue New
York,
and 106th Street New
York
10029
Heferences 1. O'BRIEN, J, E., and STOUT, A. P.: hIalignant fibrous xanthomas, Cancer 17: I445 (1964). 2. STOUT, A. P.,and LATTES, R.: Tllmorsof Soft Tissues, Atlas of Tumor Pathology, Washington, D.C., Armed Forces Institute of Pathology, 1966,p.38.
461