ATYPICAL DESMOID TUMOR OF THE ABDOMEN: A CASE REPORT
3
ATYPICAL DESMOID TUMOR OF THE ABDOMEN: A CASE REPORT Anthony C. Disher, MD, Mahua Biswas, MD, Theodore Q. Miller, MD, and Amos Kuvhenguhwa, MD Los Angeles, California Desmoids are rare lesions that are histologi- cally benign but locally aggressive. These lesions should be considered in patients who present with abdominal masses, particularly if there is a prior history of familial polyposis. This case report describes a patient with an abdominal desmoid that demonstrated atypical features on computed tomography and correla- tive magnetic resonance images. (J Nati Med Assoc. 1993;85:309-31 1.) Key words * abdominal desmoid * Gardner's syndrome Abdominal desmoids are rare and not extensively discussed in the radiologic literature. As of 1989, less than 2000 cases had been reported in the literature.' The largest series of patients studied with computed tomography (CT) was reported by Einstein et al.2 These lesions may occur in a variety of locations, including the mesentery, retroperitoneum, and abdominal wall. Initial diagnosis is most commonly made with CT scanning, which also may be used to monitor the response of the lesions to therapy. This article describes an atypical case of abdominal desmoid. CASE REPORT An 18-year-old mentally retarded male was admitted with complaints of weight loss, vomiting, and suprapu- bic mass of approximately 6 months duration. Physical examination revealed an emaciated patient with a large, firm, tender suprapubic mass. There was no known history of familial polyposis or other conditions that From the Departments of Radiology and Surgery, King/Drew Medical Center, Los Angeles, California. Requests for reprints should be addressed to Dr Anthony C. Disher, Dept of Radiology, King/Drew Medical Ctr, 12021 S Wilmington Ave, Los Angeles, CA 90059. predispose to desmoid tumor. Laboratory studies showed normal electrolytes and renal function tests but a low serum albumin level. A plain radiograph of the abdomen demonstrated a large lower abdominal/upper pelvic mass causing superior displacement of adjacent bowel loops (Figure 1). A CT scan and magnetic resonance imaging (MRI) of the abdomen and pelvis were obtained for further characterization of the mass. The CT scan demonstrated a mass of primarily soft tissue density with a central area of low attenuation and several calcifications (Figure 2). The mass appeared to arise from the mesentery. There was no evidence of intra-abdominal or retroperitoneal adenopathy. The Tl -weighted co- ronal MR image demonstrated a mass of intermediate signal intensity causing displacement of both the common and internal iliac arteries (Figure 3). The mass was of inhomogeneous signal intensity on the T2- weighted images (Figure 4). An abdominal aortogram was requested by the consulting surgeon and demon- strated arterial supply primarily from both internal iliac arteries (Figure 5). The mass caused displacement of the inferior mesenteric artery, as well as both the common and internal iliac arteries. Common clinical complica- tions, such as hydronephrosis or small bowel obstruc- tion, were not present. The differential diagnoses for this mass include teratoma, leiomyoma, sarcoma of undetermined histogenesis, and abdominal desmoid. At laparotomy, a mass with a pedicle from the lower greater omentum was observed (Figure 6). A wide surgical resection was performed. No mesenteric adeno- pathy, ascites, or peritoneal implants were identified. The gross specimen measured 12.6 cm X 12 cm. Section of the mass revealed a desmoid-type fibrous tumor with no true capsule. A pathologic diagnosis of desmoid tumor of the abdomen was reported. The patient tolerated the surgical excision of the mass without complication and was discharged home to the care of relatives. JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 85, NO. 4 309
Transcript of ATYPICAL DESMOID TUMOR OF THE ABDOMEN: A CASE REPORT
ATYPICAL DESMOID TUMOR OFTHE ABDOMEN: A CASE REPORTAnthony C. Disher, MD, Mahua Biswas, MD, Theodore Q. Miller, MD, andAmos Kuvhenguhwa, MDLos Angeles, California
Desmoids are rare lesions that are histologi-cally benign but locally aggressive. Theselesions should be considered in patients whopresent with abdominal masses, particularly ifthere is a prior history of familial polyposis.This case report describes a patient with anabdominal desmoid that demonstrated atypicalfeatures on computed tomography and correla-tive magnetic resonance images. (J Nati MedAssoc. 1993;85:309-31 1.)
Key words * abdominal desmoid * Gardner's syndrome
Abdominal desmoids are rare and not extensivelydiscussed in the radiologic literature. As of 1989, lessthan 2000 cases had been reported in the literature. ' Thelargest series of patients studied with computedtomography (CT) was reported by Einstein et al.2 Theselesions may occur in a variety of locations, includingthe mesentery, retroperitoneum, and abdominal wall.Initial diagnosis is most commonly made with CTscanning, which also may be used to monitor theresponse of the lesions to therapy. This article describesan atypical case of abdominal desmoid.
CASE REPORTAn 18-year-old mentally retarded male was admitted
with complaints of weight loss, vomiting, and suprapu-bic mass of approximately 6 months duration. Physicalexamination revealed an emaciated patient with a large,firm, tender suprapubic mass. There was no knownhistory of familial polyposis or other conditions that
From the Departments of Radiology and Surgery, King/DrewMedical Center, Los Angeles, California. Requests for reprintsshould be addressed to Dr Anthony C. Disher, Dept ofRadiology, King/Drew Medical Ctr, 12021 S Wilmington Ave,Los Angeles, CA 90059.
predispose to desmoid tumor. Laboratory studiesshowed normal electrolytes and renal function tests buta low serum albumin level. A plain radiograph of theabdomen demonstrated a large lower abdominal/upperpelvic mass causing superior displacement of adjacentbowel loops (Figure 1).A CT scan and magnetic resonance imaging (MRI) of
the abdomen and pelvis were obtained for furthercharacterization of the mass. The CT scan demonstrateda mass of primarily soft tissue density with a centralarea of low attenuation and several calcifications(Figure 2). The mass appeared to arise from themesentery. There was no evidence of intra-abdominalor retroperitoneal adenopathy. The Tl -weighted co-ronal MR image demonstrated a mass of intermediatesignal intensity causing displacement of both thecommon and internal iliac arteries (Figure 3). The masswas of inhomogeneous signal intensity on the T2-weighted images (Figure 4). An abdominal aortogramwas requested by the consulting surgeon and demon-strated arterial supply primarily from both internal iliacarteries (Figure 5). The mass caused displacement of theinferior mesenteric artery, as well as both the commonand internal iliac arteries. Common clinical complica-tions, such as hydronephrosis or small bowel obstruc-tion, were not present. The differential diagnoses forthis mass include teratoma, leiomyoma, sarcoma ofundetermined histogenesis, and abdominal desmoid.
At laparotomy, a mass with a pedicle from the lowergreater omentum was observed (Figure 6). A widesurgical resection was performed. No mesenteric adeno-pathy, ascites, or peritoneal implants were identified. Thegross specimen measured 12.6 cm X 12 cm. Section of themass revealed a desmoid-type fibrous tumor with no truecapsule. A pathologic diagnosis of desmoid tumor of theabdomen was reported. The patient tolerated the surgicalexcision of the mass without complication and wasdischarged home to the care of relatives.
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 85, NO. 4 309
ATYPICAL DESMOID
Figure 1. A plain abdominal film demonstrated. a. mass in the:.ower abdo.men/upprpelvis. Figure 2. Contiguous transaxial CT imaages revealed a softtiss.ue. .mass with.central necrosis.and scattered calcification.s. FIgure 3. Tl -weighted coro'nal MR image:demonstrates: a mass of intermediate signal ntnsint displacing both the coon andinternal iliac arteries. Figure 4. T2-weighted axial M:R image demonstrated a: peM massof inhomogeneous signal intensity. Figure 5." An abdominal aortogram reealed:thevascular supply primarily from branches of both internal iliac arteries. :Figur 6. Thegross specimen removed at surgery -was attached to the inferior portion f the geateromentum.
...~~~~~~~~~~~~~~~~~~. .. . .... ....;..~~~~~~~~~~~~~~~~~~~~................... .. . ~~~~~~~~~~~~~~~~~~~~~....
.........}-~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~ .. .. . .,................... ....................... .....
- X.0.0]~~~~~~~~~~~~~~~~~~~~. -..
.......
DISCUSSIONPrimary peritoneal, omental, and mesenteric tumors
are rare. Solid omental and mesenteric tumors havesimilar pathologic features and may arise from anyelements found in these structures. Solid peritonealtumors can occur at any age and can be either benign ormalignant.3'4
Abdominal desmoid tumors arise from fibrouselements within the mesentery or interior abdominalwall musculature. Histologically, desmoids are part of alarger group of benign fibrous lesions, fibromatosetumors, which do not metastasize.5 Therefore, desmoidsare classified as benign lesions.6 Although these tumorscan occur as isolated lesions, they have been reported inassociation with familial polyposis and postcolectomyin patients with Gardner's syndrome. The tumor is morecommon in women, with a female:male ratio of 3: 1, and
the etiology is unknown.7 Desmoid tumors are notencapsulated and vary widely in size.
Patients have few symptoms until the tumor reachesa size large enough to cause abdominal fullness, apressure sensation, or partial bowel obstruction. Thetumor can locally invade adjacent structures. Localrecurrence occurs in 50% of patients despite radicalsurgical therapy. Wide surgical resection is the treat-ment of choice. Radiation therapy and/or chemotherapydemonstrate no benefit.8
Radiographically, desmoid tumors appear as abdomi-nal masses that displace adjacent bowel loops. Mesen-teric infiltration may be seen on barium examinations asretraction, angulation, and distortion of small or largebowel. Computed tomography images typically demon-strate desmoid tumors as solid, well-circumscribedmasses of soft tissue density that do not contain
310 JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 85, NO. 4
ATYPICAL DESMOID
calcium. Our patient presented with a mass containinga central area of low attenuation and associatedcalcifications.A desmoid tumor has low signal intensity on
TI-weighted MRI and remains low in intensity onT2-weighted images. This finding is caused by thefibrous content of the tumor.9 The desmoid tumordescribed here demonstrated intermediate signal inten-sity on Ti-weighted images and inhomogeneous signalintensity on T2-weighted images. On ultrasonography,these tumors are fairly well demarcated masses, havingoccasional dense internal echoes.7
Angiography is important in planning surgery toknow if the tumor affects the omentum primarily orsecondarily. I0
Literature Cited1. Easter DW, Halasz NA. Recent trends in the manage-
ment of desmoid tumors: summary of 19 cases and review ofthe literature. Ann Surg. 1989;210:765-769.
2. Einstein DM, Tagliabue JR, Desai RK. Abdominaldesmoids: CT findings in 25 patients. American Journal of
Radiology. 1991;157:275-279.3. Gonzalez-Crussi F, Sotelo-Avila C, deMello DE. Primary
peritoneal, omental, and mesenteric tumors in childhood.Semin Diagn Pathol. 1986;3:122-137.
4. Weinberger HA, Ahmed MS. Mesenchymal solid tumorsof the omentum and mesentery: report of four cases. Surgery.1 977;82:754-759.
5. Reitamo JJ, Scheinin TM, Hayry R The desmoidsyndrome: new aspects in the cause, pathogenesis andtreatment of the desmoid tumor. Am J Surg. 1 986;1 51:230-237.
6. Kissane JM. Anderson's Pathology. 9th ed. St Louis, Mo:CV Mosby Co; 1990.
7. Stephens D, ed. Gastrointestinal Disease (Fourth Se-ries) Test and Syllabus. Reston, Va: The American College ofRadiology; 1990.
8. Schwartz RW, Reames M, McGrath PC, Letton RW,Appleby G, Kenady DE. Primary solid neoplasms of the greateromentum. Surgery. 1991;100:543-549.
9. Lee JKT, Sagel SS, Stanley RJ. Computed BodyTomography With MRI Correlation. New York, NY: RavenPress; 1989.
10. Aspestrand F, Lexow P, Andersen JIA. The angiogra-phic features of tumor infiltration of the greater omentum.Radiologe. 1985;25:488-489.
You can help us raise the cure rateforcolorectal cancer.
........_"If everyone over 50 had
n 1 _l ~~~~~~~~checkups for colorectal cancer,| i1 i | _ | ~~~~~~thecure rate could be as highi > > l l ~~~~~~~as 75%," says Dr. LaSalle D.
|ziyli _ j ~~~~~~~~Leffall, Jr., past president,.............American Cancer Society "You
- _ ~~~~~~can'tcure it if you don't know
perform the digital and
vii~~~~~~~~~~~tk c, are of the simpl toColorectal cancer warning
signs are a change in bowelhabits and blood in the stool.
; is Checkup Guidelines forAmen and women over 50can'withou tsymptoms:
__ a l~ ~~~~~~~stool blood test annuallyFFs ~~~~~ * ~~procto exam every 3 to 5.s> > 11 - _ = ~~~~~years after 2 negative tests^_ ~~~~~~~~~1 year apart.
perform thcancer alone.
JOURNALOF.TNNELO
JOURNAL OF THE NATIONAL MEDICALASSOCIAcare,ofOthe8simple stool
