Desmoid tumors of the abdominal wall

DESMOID TUMORS

OF THE ABDOMINAL

WALL

JOSEPHGASTER, M.D. Los Angeles,

ESMOID tumors are of interest beare uncommon and cause they frequently are a probIem in differentia1 diagnosis. These tumors are important because they can be excised surgically uith permanent cure if a11 of the tumor is removed. MiIIer (I 838) first used the word “desmoid” to describe these benign tumors which arise from musculoaponeurotic structures. The word is we11 chosen as it represents a contraction of two Greek words, “desmos,” a band or tendon, and “eidos,” appearance. Desmoids may deveIop in any of the striated muscIes or their aponeuroses, for instance, in the pectorahs major, sternoCIeidomastoid, digastricus, masseter muscles, scapular region, biceps, extensor carpi uInaris, rectus femoris, gIutei or hamstring muscIes. They do not appear in these regions, however, as frequentIy as they are found in the abdomina1 waI1. This paper deals onIy with desmoids of the abdomina1 wall. In this region they occur most frequentIy in reIation to the rectus muscle beIow the IeveI of the umb&us. Stone* found the right Iower part of the abdomina1 wall invoIved more frequently than any other portion of the abdomen. Various authors2,j-’ give different occurrence rates. ApproximateIy 90 per cent of the reported cases were in women and about 90 per cent of these women had borne chiIdren. AIthough the exact etioIogic factor is unknown, Waughs summarizes severa observations which seem to indicate that these fibromas resuIt from trauma with hemorrhage; and that the characteristics of a tumor are assumed during the

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process of organization of the hematoma. The folIowing observations bear this out: (I) frequent history of trauma to the region; (2) the tumors have occurred in the scars of previous incisions and (3) they are most common among women who have been pregnant. Pathology. The d esmoids are denseIy hard and cut with a grating sound. The cut surface reveals interIacing bands of fibrous tissue. The Iarger tumors tend to be soft in the center and some are actuaIIy cystic. AIthough StraublO reports changes in the overIying skin and keloid fbrmation, this is distinctIy unusua1. MicroscopicaIIy, the appearance is that of a ceIIuIar fibroma occurring in striated muscIe. Ewing3 states that not infrequently the structure resembIes a neurofibroma and varies from a hard, aceIIuIar fibroma to a rather celIuIar fibrosarcoma. GeneraIIy speaking, desmoids are not maIignant and, whiIe prone to IocaI recurrence, do not metastasize. Sarcomas shouId not be included in this group. The center of the tumor is oider than the periphera1 portion. At the periphery the tumor frequently infiItrates the surrounding muscIe. Waugh and others state that there is no capsule or definite line of cIeavage between the tumor and the adjacent muscle. When the tumor is adjacent to a fascial plane, an appearance of encapsulation may resuIt. The inclusion of striated muscle fibers is an unusua1 feature of the tumor. From the practica1 surgica1 standpoint it is undoubtedIy easy to Ieave infiItrating portions

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Gaster-Desmoid

9oo

Tumors

been successfu1 in curing these tumors but may be used as a suppIement to surgery. This is particuIarIy desirabIe when compIete remova of the tumor is impossibIe. RemovaI of large tumors may Ieave a defect in the abdomina1 waI1 that is difhcuIt to cIose and which may require fascia1 transpIants CASE REPORTS

The foIIowing patients on whom we operated present some interesting features.

FIG. I. Gross appearance rectus muscle.

of desmoid

tumor

of right

of the tumor in the surrounding muscuIature. Pearman and Mayo’s5 recent study has shown that these tumors are definitely benign and that “recurrences” are actuaIIy the resuIt of incompIete remova1. They found that patients with these “recurrences,” when * subsequentIy undergoing compIete excision, were cured for years. History and Examination. UsuaIIy the patient finds a smaI1, hard Iump in the Iower abdomina1 waI1 or she may have been totaIIy unaware of the mass which is discovered during the course of a routine physica examination. When the mass gets larger, it may be visibIe (Smith4) or may produce a dragging sensation and occasionaIIy actua1 pain due to the weight of the tumor. Waiters and Church’l found that onIy one-fourth of the patients in their series compIained of pain or soreness. Examination reveaIs a mass fixed in the abdomina1 waI1 which does not move with respiration. The mass can be moved to a sIight degree with the abdominal muscIes relaxed. With these muscIes tensed, however, the tumor is compIeteIy immovabIe. The tumor may seem more superfIcia1 than the usua1 intraperitonea1 mass. It is not attached to the skin, thus distinguishing it from a keIoid. Treatment. The treatment is wide surgica1 excision. Radiotherapy aIone has not

CASE I. The patient was a thirty-six year oId white fernare who compIained of pain in the right side of her abdomen of approximateJy two weeks’ duration. The past history revealed that she was married for eIeven years and had two chiJdren. One child was nine years oId and the second chiId was two. The remainder of the past history was irreIevant. About two weeks before seeking medica advice the patient noticed the gradua1 onset of an aching type of pain in the right side of the abdomen. The pain was continuous and did not radiate. It was more severe in the right upper quadrant than in the right lower quadrant. There was no history of indigestion or seJective dyspepsia and the pain was unaffected by food intake. Examination reveaIed a short, stocky woman weighing 220 pounds. The abdomen was very obese. A gJobuIar, firm, tender mass 6 to 8 cm. in diameter was paJpabJe in the right upper quadrant. AIthough there was moderate spIinting of the abdomen on the right side, the Jiver edge couJd be feIt about two fingers beIow the Costa1 margin. Tenderness made deep paIpation of the mass diffIcuIt. The mass descended sIightIy with respiration. The bIood count was as foIIows: red bIood ceIIs, 4,goo,ooo; hemogIobin, 13 Gr+, 82 per cent; white bIood ceIIs, 12,800; neutrophiIes, 70; segmented poJymorphonucIears, 65; stab ceIJs, 3; Iymphocytes, 22; monocytes, 6; basophiIes 2; shift to the Ieft. AIthough the mass was more medial and firmer than usua1, before x-rays were taken a tentative diagnosis of hydrops or carcinoma of the gaIIbIadder was made. The patient was hospitaIized. Temperature on admission was gg%., puIse 80. Cholecystography reveaIed a normaIIy functioning gaIIbIadder without stones. Urine examination showed a trace of albumin. American

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Gaster-Desmoid

Tumors

FIG. 3. Photomicrograph of edge of desmoid showing invasion of rectus muscle.

FIG. z. High power magnification showing transformation from normal aponeurosis on the right to interlacing bundles of desmoid tumor on the left.

In view of the negative x-ray findings, expforatory operation was decided upon in order to determine the exact nature of the mass. At operation after going through a pannicmus adiposis of about IO cm. in depth, the tumor was finahy encountered. It had completely repIaced the rectus muscle for about 6 to 8 cm. A wide excision was done and it was necessary to remove portions of the anterior and posterior rectus sheaths as we11 as the muscIe. The peritoneum was opened and the liver was seen to The gaIIdescend beIow the Costa1 margin. bladder and remaining viscera were normaI. The appendix was removed and the defect in the abdominal wall was closed longitudinally in Iayers with some difhcu1ty using interrupted silk sutures. The pathologist reported that the growth removed was a desmoid tumor of the abdomina1 wall measuring 8 by 5 by 5 cm. Figure I shows the gross appearance of the tumor. Figure 2 shows a photomicrograph of the edge of the desmoid tumor showing the transition from normal fascia into interlacing bundles of desmoid tumor. Convalescence was uneventfu1. The patient was out of bed the day after surgery and went home on the sixth postoperative day. CASE II. hf. M. a twenty-seven year old December,

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white female was admitted to the Alexander BIain HospitaI, Detroit, Mich. She had noticed a Iump in her Iower abdomen on the right side six months before admission. Her past history revealed that she had been married five ,years before and had had one deIivery. One year previously she had a uterine suspension done eIsewhere through a midline suprapubic incision. The tumor had grown gradually larger during the past few months. Examination reveaIed the uterus enlarged to the size of a seven-month pregnancy. There was an irreguIar, nodmar, painIess mass measuring 2 by 7 cm. present above the inguinal ligament just to the right of the midline. The skin was movabIe over the mass. The blood count and urine examinations were normal. The patient was operated upon with a preoperative diagnosis of tumor of the abdominal waI1, possibly mahgnant. A 6 inch incision was made and the tumor was widely excised incIuding generous portions of the invoIved fascia. A cIassicaI cesarian section was then done. Due to the large defect the abdomina1 waI1 was cIosed with some diffrcuIty. The pathoIogist described a desmoid tumor measuring IO by 4 by 4 cm. (Fig. 3.) The patient’s postoperative course was uneventful. Eighteen months and eight months, respectively, since the dates of surgery both wounds were we11 hea1ed without any sign of recurrence or hernia.

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Gaster-Desmoid COMMENT

Whereas most cases of desmoid tumors are painIess, it is interesting to note that the first patient had pain and tenderness. The physica signs simulated a hydrops or carcinoma of the gaIIbIadder or an inff amed gaIIbIadder with omentum wrapped around it. The difficuIty of differentiating an intraperitoneal mass from a mass in the depths of the abdomina1 waI1 by physica examination was insurmountabIe due to the patient’s obesity. At operation the mass was seen to be about IO cm. below the Ievel of the skin whereas the gaIlbIadder was in direct contact with the deep surface of the tumor. The second case is of interest for two reasons. A maIignant-appearing tumor proved to be benign and was cured by compIete radical excision. Cesarian section could be done at the same time. SUMMARY

Desmoid tumors are frequentIy preceded by some form of trauma. These tumors are

Tumors benign and wiI1 not recur if excised wideIy. A case is reported which simuIated hydrops of the gaIlbIadder and another which simuIated maIignant tumor of the abdominaI waI1. REFERENCES I. MASSON, J. B. Desmoid tumors. Ann. Surg., gz: 444. 1930. 2. PFEIFFER, C. Beitr. z klin. Cbir., 44: 334, 1904. 3. EWING, J. NeopIastic Diseases. 4th ed. PhiIadelphia, 1040. ,_ W. B. Saunders Co. 4. SMITH, M. K. Christopher’s Textbook of Surgery. 4th ed. Philadelphia, 1945. W. B. Saunders Co. ,L PEARMAN. R. 0. and MAYO. C. W. Desmoid tumors; cIinica1 and pathoIogic study. Ann. Surg., I 15: 114-125, 1942. 6. JUDD, D. B. and MASSON, J. C. Desmoid tumor. Minnesota Med., 27: qg-280, 1944. 7. GURTT, Quoted by REPETTO, E. Fibroma deIIa parete addominale anteriore. Policlinico (ser. cbir.), 41: 564-578, 1934. 8. STONE, H. B. Ann. .%rg., 48: 175, 1908. 9. WAUGH, J. M. Fibroma of the muscuIofascia1 Iayers of the abdomina1 waI1 (desmoid tumors). 50: 694, ‘940. IO. STRAUB, G. F. Desmoid tumors; report of case. California @ West. Med., 31: I86-rgo, 1929. I I. WALTERS, W. and CHURCH, G. T. Desmoid tumor of left rectus muscIe. S. Clin. North America, 14: 647-649,

1934.

American

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of Surgery