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1031

Primary Malignant Small Round Cell Tumor of the Abdomen: CT Findings

in Five Cases

Malignant small round cell tumor of the abdomen is a newly described clinicopathoDatla G. K. Varma1 logic entity. The features of this entity include a predilection for young men, predominant Kelvin McDaniel1 location, and aggressive behavior. Pathologic analysis demonstrates a Nelson G. Ord#{244}#{241}ez2 abdominopelvic small-cell tumor unusually coexpressing desmin and keratin. This study describes CT Christine A. J. Granfiel& characteristics in five patients with malignant small round cell tumor of the abdomen. Chusilp Charnsangavej1 All patients were young white men, 20-36 years old (mean, 28 years). In all five cases, Sidney Wallace1 CT

scans

surfaces

revealed

lobulated

and predominantly

and pelvis

(two cases);

masses were centrally iodinated

contrast

masses

appearing

the

involving

diffuse abdominopelvic necrotic and enhanced

material.

Additional

to arise

primarily from peritoneal of the abdomen (two cases) involvement was noted in one case. The inhomogeneously after administration of

peritoneal

cavity

CT features

included

ascites

(one case),

calcifi-

cations in the dominant mass (one case), omental implants (two cases), hydronephrosis (four cases), anterior diaphragmatic lymphadenopathy (two cases), liver metastases (two cases), and retroperitoneal lymphadenopathy (two cases). CT findings of abdominal malignant small round cell tumor are nonspecific and mimic findings noted in other conditions, such as pentoneal carcinomatosis and leiomyosarcomatosis. AJR

158:1031-1034,

May

1992

A distinctive type of malignant small round cell tumor of the abdomen has been recently described in the pathologic literature [1-4]. Unique features of this neoplasm include a predilection for young men, predominant location in the peritoneal cavity with a possible predilection for the pelvic region, and aggressive behavior with high mortality despite multiple modes of therapy [2]. Immunohistochemical studies are unique in this entity in that there is coexpression of keratin and desmin indicating the presence of epithelial and muscle-associated antigens [1 2]. To our knowledge, malignant small round cell tumor of the abdomen has not been previously reported in the radiologic literature. Abdominal CT findings in five patients with malignant small round cell tumor of the abdomen are the subject of this report. ,

Materials

and

We searched Received revision

November 13, 1991 December 16, 1991.

;

accepted

after

2

Department

of Pathology, Cancer

The

0361-803X/92/1 585-1031 CAmerican

Roentgen

University

of

Center, Houston, TX

77030. Ray SOciety

with

abdomen

I Department of Diagnostic Radiology, Box 57, The University of Texas M. D. Anderson Cancer Center, 1515 HOICOmbO Blvd., Houston, TX 77030. Address reprint requests to D. G. K. Varma.

Texas M. D. Anderson

patients

Methods

the consultation

malignant

and pelvis had

files of one of the authors

small

round

cell tumor

been

obtained.

and found the records

of the abdomen

in whom

The CT scans were reviewed

CT scans

retrospectively.

of five of the

All five

patients were young white men 20-36 years old (mean, 28 years). The patients had no clinical evidence of AIDS. Two patients tested negative for antibodies to HIV; the other patients were not tested. The patients had palpable abdominal and/or pelvic masses. One patient

also

had an enlarged

metastatic

left anterior

cervical

lymph

node.

Signs

and

symptoms

were related to mass effect on contiguous organs. Diagnosis was based on pathologic examination of tissue specimens obtained during exploratory laparotomy (four cases) and from a resected

cervical

lymph

was done by one of the authors,

node (one case).

The histopathologic diagnosis of this tumor small round cell tumor of the

who is familiar with malignant

1032

VARMA

abdomen

[1].

Immunohistochemical

studies

were

performed

in all

cases by using the avidin-biotin-peroxidase complex method [5]. The details of additional immunostaining procedures performed are de-

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scribed

elsewhere

[6]. Tissue

for electron

microscopy

studies

was

available in one case. CT scans were obtained with a GE 9800 scanner(General Electric, Milwaukee, WI) in three cases and with GE 8800 and Picker 1200 SX (Picker International, Highland Heights, OH) scanners in one case each. The scans consisted of contiguous 1 .0-cm-thick sections. In all patients scans were obtained after IV infusion of 150 ml of contrast material and with oral and rectal administration of diluted barium. In three

patients,

injector

IV contrast

material

was

administered

at 1 .5 mI/sec with a 60-sec delay; in

drip infusion

technique

was used.

two

In two patients,

AJR:158,

May 1992

tures included ascites (one case), omental implants (two cases), hydronephrosis (four cases), anterior diaphragmatic lymphadenopathy (two cases), liver metastases (two cases), and retroperitoneal lymphadenopathy (two cases)(Figs. 2 and 4). The hydronephrosis was caused by the pelvic masses impinging on the ureters; the masses also abutted loops of bowel causing symptoms of intermittent bowel obstruction.

Pathology

via power

patients,

a rapid

unenhanced

scans

were also obtained. The CT scans were analyzed for anatomic location of tumor, morphologic characteristics, extent of disease, and associated findings.

Results In all five cases, CT scans revealed lobulated masses appearing to arise from peritoneal surfaces and ranging in size from 5 x 4 cm to 1 5 x 8 cm. In two cases, the masses were located predominantly in the peritoneal cavity of the left abdomen extending inferiorly from the greater curvature of the stomach to the level of the lower pole of the kidneys (Figs. 1 and 2). The masses were centrally necrotic and enhanced inhomogeneously after administration of iodinated contrast material; one tumor demonstrated foci of calcification (Fig. 2). A smaller pelvic component posterior to the urinary bladder was also noted in one of these tumors. In two patients, large lobulated masses were identified in the pelvis posterior to the bladder and anterior to the rectum (Fig. 3), and one patient had diffuse abdominopelvic involvement. Additional CT fea-

A

ET AL.

Histologically, the five tumors were similar (Fig. 5). They were made up of well-defined, solid clusters of small tumor cells separated by dense fibrous stroma. The larger tumor nests had central necrosis, and mitotic figures were frequent. lmmunocytochemical studies demonstrated that the tumor cells stained with keratin in all cases; in addition to keratin, most tumor cells also reacted with desmin and vimentin. No reactivity for chromogranin A or S-i 00 protein was seen in any of the cases. The most striking ultrastructural feature was the presence of large amounts of intracytoplasmic intermediate filaments that often formed paranuclear bundles and whorls. No neuroendocrine granules were seen.

Discussion Malignant small round cell tumor of the abdomen appears to be a distinct clinicopathologic entity belonging to the generic category of small round cell tumors of infancy and childhood [2, 7, 8]. The category of small round cell tumors of infancy and childhood includes Ewing’s sarcoma, primitive neuroectodermal tumor, embryonal and rhabdomyosarcoma,

B Fig. 1.-32-year-old

A, Contrast-enhanced abdomen. B, CT scan obtained

man with Increasing abdominal girth and palpable abdominal CT scan shows large lobulated mass with central necrosis

mass.

at lower level of mass shows small omental nodules (arrows). mass extended to greater curvature of stomach.

in left side

Fig. 2.-27-year-old of

trast-enhanced Supenoriy,

man with signs and symptract obstruction. Conaxial CT scan of 1-cm-thick section

toms of bowel and urinary

shows lobulated, septated, centrally necrotic mass that extended inferioriy from greater curvature stomach. A smaller polvic mass was the cause

hydronephrosis. A small focus of calcification was also noted on an unenhanced scan.

row)

of of (ar-

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AJA:158,

Fig.

CT OF ABDOMINAL

May 1992

3.-24-year-old

man

with

palpable

Fig. 5.-A, small

Low-power

photomicrograph

round

neoplasm

cell

shows

ROUND

CELL

TUMOR

1033

Fig. 4.-36-year-old man with tumor progression despite multidrug therapy. A, Contrast-enhanced CT scan shows liver metastases, lnteraortocaval retroperitoneal lymphadenopathy (large arrow), and abdominal implants (small arrow). B, CT scan obtaIned at superior aspect of liver shows anterior diaphragmatic lymphadenopathy (arrow) In addition to liver metastases.

pelvic

mass and enlarged left anterior cervical nodes. Contrast-enhanced CT scan shows large lobulated, centrally necrotic pelv’ic mass and dilated left ureter (arrow).

lignant

SMALL

of matumor

nest surrounded by dense stroma. (H and E, origInal magnification x120) B, Photomicrograph

of

immunocytochemical

preparation shows reactivity for keratin throughout cytoplasm of tumor cells. (Avidin-biotin-peroxidase complex stain, original magnification x300)

lymphoma, and neuroblastoma [2]. The most characteristic diagnostic feature of this tumor is the coexpression of epithehal (keratin) and mesenchymal types of intermediate filaments (desmin and vimentin). This unique immunophenotype, together with a marked predilection for young men, abdominopelvic location, and aggressive clinical behavior, supports the concept that malignant small round cell tumor of the abdomen represents a distinct clinicopathologic entity [1 2]. The exact cell of tumor origin is not known, but in most instances, it appears to arise from the peritoneum [1 2]. Experience in the treatment of malignant small round cell tumor of the abdomen is limited, and in our cases, no significant response was noted on multidrug chemotherapy regimens. The role of debulking surgery is yet to be determined. The peritoneum is more often involved by metastatic disease than it is the site of origin of primary neoplasms. The common malignant lesions that cause peritoneal carcinoma,

,

tosis are ovarian, colonic, and gastric carcinomas [9]. CT signs of peritoneal carcinomatosis include ascites, nodular peritoneal thickening, omental implants, and calcified peritoneal implants [9]. Nodular or masslike peritoneal involvement may also be seen in lymphoma, peritoneal leiomyosarcomatosis, and peritoneal mesothelioma [9-1 1 ]. Gastric leiomyosarcomas may be visualized on CT scanning as large necrotic exogastric masses extending into the abdominal cavity [12, 13]. The CT findings noted in our small series of patients with primary malignant small round cell tumor of the abdomen appear nonspecific and mimic findings noted in peritoneal carcinomatosis, leiomyosarcomatosis, and gastric leiomyosarcomas. Percutaneous fine-needle aspiration biopsy will probably be useful in the diagnosis of malignant small round cell tumor of the abdomen as pathologists become increasingly familiar with this entity. It is extremely important for

1034

VARMA

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radiologists to be aware of this newly described entity. This knowledge would help the radiologist to offer an appropriate differential diagnosis when multiple peritoneal masses are noted on abdominal CT scans, especially in young men.

REFERENCES 1 . Ordb#{241}ezNG, Zirkin A, Bloom RE. Malignant small-cell epithelial the peritoneum coexpressing mesenchymal-type intermediate Am J Surg Pathol 1989;13:413-421

2. Gerald WL, Miller HK, Battifora abdominal distinctive individuals.

tumor of filaments.

H, Mieltinen M, Silva EG, Rosai J. Intra-

desmoplastic small round-cell tumor: report of 1 9 cases of a type of high-grade polyphenotypic malignancy affecting young Am J Surg Pathol 1991;15:499-513

3. Variend 5, Gerrard M, Norris PD, Goepel JR. lntraabdorninal dermal tumour 1991;18:45-51

4. Gonzalez-Crussi

of childhood

with divergent

F, Crawford

plastic small cell tumors three cases of childhood.

SE, Chen-Chih

differentiation.

neuroecto-

Histopathology

JS. Intra-abdominal

desmo-

with divergent differentiation: observations Am J Surg Pathol 1990;14:633-642

on

New Format

ET AL.

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5. Hsu SM, Raine L, Fanger H. Use of avidin-biotin-peroxidase in immunoperoxidase

beled antibody

technique:

a comparison

(PAP) procedures.

J

between

Histochem

May 1992

complex (ABC) ABC and unla-

Cytochem

1981;29:

577-580

6. Ord#{244}hez NG, Manning

JT, Brooks

TE. Effect of trypsinization

on the

irnmunostaining of formalin-fixed, paraffin-embedded tissues. Am J Surg Pathol 1988;12:121-129 7. Unis EJ. Ewing’s sarcoma and related small round cell neoplasrns in children. Am J Surg Pathol 1986;10[suppl 1]:54-62 8. Triche TJ. Diagnosis of small round cell tumors of childhood. Bull Cancer

(Paris) 1988;75:297-310 9. Walkey MM, Friedman AC, Sohotra P, Radecki PD. CT manifestations pentoneal carcinornatosis. AJR 1988;150: 1035-1 041

of

10. Chol BI, Lee WJ, Chi JG, Han JK. CT manifestations of peritoneal Ieiomyosarcomatosis. AJR 1990;155:799-801 1 1 . Reuter K, Raptopoulos V, Reale F, et al. Diagnosis of peritoneal mesothelioma: computed tomography, sonography, and fine-needle aspiration biopsy. AJR 1983;140: 1189-1194 1 2. Nauert TC, Zomoza J, Ordonez N. Gastric Ieiomyosarcornas. AJR 1982;139:291 -297 13. McLeod IJ, Zornoza J, Shirkhoda A. Leiomyosarcorna: computed tomography findings. Radiology 1984;152: 133-1 36

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Primary malignant small round cell tumor of the abdomen: CT findings in five cases.

Malignant small round cell tumor of the abdomen is a newly described clinicopathologic entity. The features of this entity include a predilection for ...
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