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Desmoid tumor of the scapular region
Desmoid
Tumor
of the Scapular
JAMES BYRON CARTY, M.D., Diplomate, American
Region
Board of Surgery, Philadelphia, Pennsylvania herent to the IateraI edge of the scapuIa at the junction of the middIe and Iower thirds. It was covered with a dense capsuIe which was fairIy we11 demarcated except on its superior and inferior aspects where it seemed to be contiguous with the surrounding fascia. The tumor and a portion ofThe scapuIa were resected. Gross pathoIogic examination showed that the tissue was firm, gritty, buIged on section and had invaded beyond the capsuIe in two areas. Histologic examination after frozen section suggested a spindIe ceI1 sarcoma. Wider resection was therefore performed. Portions of the scapuIar muscIes surrounding the tumor were then excised together with the inferior haIf of the infraspinous portion of the scapula. ConvaIescence was uneventfu1 and the patient was discharged from the hospita1 on the tenth postoperative day. PathoIogic examination proved the fina diagnosis to be a desmoid tumor. (Figs. I and 2.) Two years after treatment a painfu1, smaI1, hard mass occurred along the IateraI edge of the scapuIa. Chest x-ray showed no evidence of metastatic disease. On May 28, 1951, this noduIe, smaIIer than the origina1, was excised with the surrounding muscIe, soft tissue and remaining infraspinous portion of the scapuIa. Microscopic sections of the tumor were simiIar to those of the Iesion excised previously. For two years there has been a continuous duI1 pain about the incision, but no evidence of recurrence. This distress is partiaIIy reIieved by cortisone therapy.
DESMOID tumors occur infrequentIy. The average incidence is Iess than 0.03 per cent1 in Iarge cIinics deaIing with neopIastic diseases. The most frequent site of desmoid tumors is within the anterior abdomina1 waI1 (69 per cent), but they may occur within striped muscIe eIsewhere. Because of these facts I wish to report such a tumor situated within the scapuIar muscuIature.
CASE REPORT R. B., a twenty-four year oId white woman, was admitted to the Presbyterian HospitaI ApriI 24, 1949, compIaning of a painfu1 mass on the IateraI aspect of her Ieft scapuIa. She stated that foIIowing the carrying of heavy packages under her Ieft arm in December, 1948, she was aware of pain in the region of her Ieft shouIder and at intervaIs on the posterior aspect of the Ieft arm. It was not unti1 April, 1949, that she noticed the mass. The past medica history and famiIy history were essentiaIIy negative and irreIevant to her present iIIness. On examination, positive findings were Iimited to the Iower third of the IateraI border of the Ieft scapuIa where there was an adherent, firm, non-tender mass. It was beneath the fascia, waInut-sized and moved with scapuIa but was unattached to the chest waI1. The bIood count showed hemogIobin 97.8 per cent, red ceIIs 4,800,000 and white ceIIs 6,100, with a norma differentia1. UrinaIysis was normaI. The serum caIcium was I 1.0 mg. per cent, the serum phosphorus 2.9 mg. per cent and the aIkaIine phosphatase 0.7 units. RoentgenoIogic examination made in different postures of the thorax with particuIar reference to the Ieft scapuIar area showed no evidence of tumor. FiIms of the Iungs were normaI. The preoperative diagnosis was tumor mass, etioIogy unknown, but maIignancy suspected. On ApriI 27th, under genera1 anesthesia, an obIique incision was made aIong the posterior axiIIary Iine and the Iatissimus dorsi muscIe spIit. The tumor was found to be firmIy ad-
ETIOLOGY
The cause of desmoid tumor is not known, but trauma frequentIy precedes its occurrence. The mechanism is probabIy organization of a hematoma with subsequent hyperpIasia, the exact cause of which is unknown. In fourteen of the thirty-four extra-abdomina1 desmoids studied by Musgrove and McDonaId,z the onset was definiteIy connected with trauma. The greater incidence of abdomina1 desmoid tumors in women (70 and 87 per cent)2-8 than in men 285
Desmoid
FIG. I. Photomicrograph dtmonstrating destroy voluntary muscIe fibers. X 120.
Tumor
of ScapuIar
the invariable
deveIopment
Region
of these tumzrs
to pcrmeatc,
engulf and
FIG. 2. Photomicrograph demonstrating the usuaI background of wavy fibrils, punctuated with ova1 nucIei. The ceIIuIarity is rather sparse, whiIe in other tumors it may be extremely rich giving a sarcomatous histoIogic appearance. The resembIance to an aponeurosis is striking and usually one can demonstrate a bIending with preexisting fascia. X 480.
is often attributed to injury caused by vioIent muscuIar contractions during Iabor. They occur most frequentIy during the third decade. Wiper and MiIIer3 reported a desmoid tumor occurring in the right infracIavicuIar region in a twenty-three year oId maIe who two months previousIy, had been injured by a she11 fragment. Penick,b in a review of over 500 desmoids, found that seventeen occurred in postoperative scars. Endocrine disturbances have aIso been considered as a cause. Lipschutz2 and his coworkers using estrogenic substances have produced fibromyomas in the abdomina1 waIIs of guinea pigs. These experimentaIIy produced tumors regressed in size when administration of estrogen was discontinued. Geschickter and Lewis,6 in examining a desmoid tumor of the abdomina1 waI1, found that it contained 13,000 rat units of gonadotropic substance per kiIogram of tissue. After studying severa connective tissue tumors, these authors concIuded that endocrine substances may play a part in their etioIogy.
to infiItrate muscle and adhere to adjacent bone. Over many years they attain great size. The Iargest reported weighed 17 kg.7 Montgomery and BIand8 excised one from the abdomina1 waI1 weighing 8.6 kg. OccasionaIIy maIignant transformation to a Iow-grade fibrosarcoma occurs, especiaIIy after numerous recurrences, but they do not metastasize. HistoIogicaIIy, they resembIe fibromas containing a varying degree of ceIIuIarity. CharacteristicaIIy a desmoid enguIfs striated muscIe fibers producing their gradua1 degeneration. The pectoraIis major, sternocIeidomastoid, diagastric, masseter, biceps, extensor carpi ulnaris, rectus femoris, gIutei, Ievator ani,g hamstring, muscIes of the dorsum of the foot, . and scapuIar region have been invoIved.3 DIAGNOSIS
The presence of a firm, fixed, non-tender, soIitary, uniIatera1, round or ova1 growth accompanied with pain is suggestive of a desmoid tumor. Musgrove and McDonaId2 state that fibrosarcoma, therapeuticaIIy the most important differentia1 diagnosis, differs from a desmoid in that “ (I) it tends to be Iarger, (2) is usuaIIy round, (3) softer in consistency, (4) in more than 50 per cent of cases it is encapsuIated, and in the remainder, is we11 circumscribed, (5) metastasis is common, and (6) the surface usuaIIy has a saImon pink homogeneous
PATHOLOGY
Desmoid tumors have been defined as nonencapsuIated, fibrous, neopIasms of tendonIike consistency arising from fascia and musstructures. These tumors cuIo-aponeurotic aIthough benign have invasive quaIities. They appear as soIitary, uniIatera1, firm, usuaIIy ovaI, sIowIy growing masses with a tendency 286
Desmoid
Tumor
of ScapuIar
Region
Two were cured, one controIIed but Iater Iost to foIIow-up. Of twenty-six cases of extra-abdomina1 desmoid tumors foIIowed one year or Ionger, twenty (77 per cent) recurred one or more times without evidence of sarcomatous change. Recurrences usuaIIy appeared during the first six months and aIways within two years aft& treatment.
appearance.” HistoIogicaIIy, the diagnosis of maIignancy may be based on the presence of pathoIogic mitotic figures, tumor giant ceIIs or encapsuIation and a high degree of ceIIuIarity. TREATMENT
The majority of writers advise wide radica1 excision as the treatment of choice. This is emphasized because of the highIy invasive quaIity of the tumor. Roentgen therapy is of IittIe, if any, additiona vaIue, aIthough Ewing stated that most of these tumors respond sIowIy but satisfactoriIy to irradiation. Roentgen therapy shouId be reserved for recurrences, the onIy partiaIIy resectabIe case, and for patients who refuse operation or are poor operative risks. High voItage roentgens or radium eIement pack therapy of over 5,000 roentgens are required. Pack and EhrIich’ suggested that the response of abdomina1 waI1 desmoids to irradiation may resuIt from suppression or cessation of ovarian function. They found that 1,000 to 1,500 roentgens directed to the tumor did not retard growth, but that at Ieast 5,000 roentgens with the porta incIuding the entire Iower abdomen are required to produce satisfactory contro1 of the tumor. In addition to radiation castration hormone therapy consisting of Iarge dosages of antifibromatogenic steroIs as testosterone, progesterone and desoxycorticosterone may prove of value in the onIy partiaIIy resectabIe cases.
SCMMARY
AND
CONCLUSIONS
A case of extra-abdomina1 desmoid tumor of the scapuIar region treated by radica1 excision and partia1 scapuIectomy with recurrence is reported. Reports of these tumors are extremeIy rare, but one seeing a firm, fixed, solitary, sIowIy growing tumor accompanied with duI1 pain shouId consider a desmoid tumor. Treatment recommended by most authorities is wide IocaI excision. Recurrences are frequent (77 per cent).2 These usuaIIy appear within the first six months and a11 within the first two years foIIowing treatment. REFERENCES I. PACK, G. T.
and EHRLICH, H. E. NeopIasms of anterior abdomina1 wall with special consideration of desmoid tumors; experience with 391 cases and coIIective review of literature. Surg., Gynec. & Obst., 79: 177-194, 1944. 2. MUSGROVE, J. E. and MCDONALD, J. R. Extraabdomina1 desmoid tumors; their differentia1 diagnosis and treatment. Arch. Patb., 45: 513540, 1948. 3. WIPER, T. B. and MILLER, J. M. Desmoid of anterior chest waI1. Am. J. Surg., 71: 556-557, 1946. 4. PENICK, R. M. Desmoid tumors deveIopine: in operative scars. Internet. S. Digest, 23: 325-329,
PROGNOSIS
WhiIe desmoid tumors are considered benign and without metastasis, IocaI recurrences are common and maIignant change occasiona1. Of 107 desmoid tumors foIIowed by Pfeiffe9 thirty-three (30.8 per cent) recurred. Eighteen recurred among eighty-five femaIes (21.2 per cent) and fifteen in twenty-two maIe patients (68.1 per cent). Pack and EhrIich’ cited eight recurrences in seventeen patients with desmoid tumors of the anterior abdomina1 waI1. They also found maIignant change more common in maIes and without reIation to size or duration of the tumor. Of seventeen cases tabuIated, three (one femaIe and two maIes) underwent maIignant change foIIowing two to six recurrences.
‘937. 5. GESCHICKTER, C. F. and LEWIS, D. Tumors of connective tissue. Am. J. Cancer, 25: 630-655, 1935. 6. EWING, J. NeopIastic Disease, 4th ed. PhiIadeIphia, 1941. W. B. Saunders. 7. STEWART, M. J. and MOUAT, T. B. Fibroma of abdomina1 waI1. Brit. J. Surg., 12: 355-377,1g24. 8. MONTGOMERY, E. E. and BLAND, P. B. Large desmoid tumor of the abdomina1 waI1. Am. Med. Pbila.. IO: 463, 1904. g. B~u~sc&wrc,~ A-.. Excision of Iarge desmoid tumor of pelvic outIet necessitating obIation of right Ievator ani muscIe. New York State J. Med. 50: 1005-1006, 1950. IO. PFEIFFER. C. Die Desmoide der Bauchdecken und ihre Prognose, Beitr. z. klin Cbir., 44: 334-901, 1904.
287
Tumor
of the Scapular
JAMES BYRON CARTY, M.D., Diplomate, American
Region
Board of Surgery, Philadelphia, Pennsylvania herent to the IateraI edge of the scapuIa at the junction of the middIe and Iower thirds. It was covered with a dense capsuIe which was fairIy we11 demarcated except on its superior and inferior aspects where it seemed to be contiguous with the surrounding fascia. The tumor and a portion ofThe scapuIa were resected. Gross pathoIogic examination showed that the tissue was firm, gritty, buIged on section and had invaded beyond the capsuIe in two areas. Histologic examination after frozen section suggested a spindIe ceI1 sarcoma. Wider resection was therefore performed. Portions of the scapuIar muscIes surrounding the tumor were then excised together with the inferior haIf of the infraspinous portion of the scapula. ConvaIescence was uneventfu1 and the patient was discharged from the hospita1 on the tenth postoperative day. PathoIogic examination proved the fina diagnosis to be a desmoid tumor. (Figs. I and 2.) Two years after treatment a painfu1, smaI1, hard mass occurred along the IateraI edge of the scapuIa. Chest x-ray showed no evidence of metastatic disease. On May 28, 1951, this noduIe, smaIIer than the origina1, was excised with the surrounding muscIe, soft tissue and remaining infraspinous portion of the scapuIa. Microscopic sections of the tumor were simiIar to those of the Iesion excised previously. For two years there has been a continuous duI1 pain about the incision, but no evidence of recurrence. This distress is partiaIIy reIieved by cortisone therapy.
DESMOID tumors occur infrequentIy. The average incidence is Iess than 0.03 per cent1 in Iarge cIinics deaIing with neopIastic diseases. The most frequent site of desmoid tumors is within the anterior abdomina1 waI1 (69 per cent), but they may occur within striped muscIe eIsewhere. Because of these facts I wish to report such a tumor situated within the scapuIar muscuIature.
CASE REPORT R. B., a twenty-four year oId white woman, was admitted to the Presbyterian HospitaI ApriI 24, 1949, compIaning of a painfu1 mass on the IateraI aspect of her Ieft scapuIa. She stated that foIIowing the carrying of heavy packages under her Ieft arm in December, 1948, she was aware of pain in the region of her Ieft shouIder and at intervaIs on the posterior aspect of the Ieft arm. It was not unti1 April, 1949, that she noticed the mass. The past medica history and famiIy history were essentiaIIy negative and irreIevant to her present iIIness. On examination, positive findings were Iimited to the Iower third of the IateraI border of the Ieft scapuIa where there was an adherent, firm, non-tender mass. It was beneath the fascia, waInut-sized and moved with scapuIa but was unattached to the chest waI1. The bIood count showed hemogIobin 97.8 per cent, red ceIIs 4,800,000 and white ceIIs 6,100, with a norma differentia1. UrinaIysis was normaI. The serum caIcium was I 1.0 mg. per cent, the serum phosphorus 2.9 mg. per cent and the aIkaIine phosphatase 0.7 units. RoentgenoIogic examination made in different postures of the thorax with particuIar reference to the Ieft scapuIar area showed no evidence of tumor. FiIms of the Iungs were normaI. The preoperative diagnosis was tumor mass, etioIogy unknown, but maIignancy suspected. On ApriI 27th, under genera1 anesthesia, an obIique incision was made aIong the posterior axiIIary Iine and the Iatissimus dorsi muscIe spIit. The tumor was found to be firmIy ad-
ETIOLOGY
The cause of desmoid tumor is not known, but trauma frequentIy precedes its occurrence. The mechanism is probabIy organization of a hematoma with subsequent hyperpIasia, the exact cause of which is unknown. In fourteen of the thirty-four extra-abdomina1 desmoids studied by Musgrove and McDonaId,z the onset was definiteIy connected with trauma. The greater incidence of abdomina1 desmoid tumors in women (70 and 87 per cent)2-8 than in men 285
Desmoid
FIG. I. Photomicrograph dtmonstrating destroy voluntary muscIe fibers. X 120.
Tumor
of ScapuIar
the invariable
deveIopment
Region
of these tumzrs
to pcrmeatc,
engulf and
FIG. 2. Photomicrograph demonstrating the usuaI background of wavy fibrils, punctuated with ova1 nucIei. The ceIIuIarity is rather sparse, whiIe in other tumors it may be extremely rich giving a sarcomatous histoIogic appearance. The resembIance to an aponeurosis is striking and usually one can demonstrate a bIending with preexisting fascia. X 480.
is often attributed to injury caused by vioIent muscuIar contractions during Iabor. They occur most frequentIy during the third decade. Wiper and MiIIer3 reported a desmoid tumor occurring in the right infracIavicuIar region in a twenty-three year oId maIe who two months previousIy, had been injured by a she11 fragment. Penick,b in a review of over 500 desmoids, found that seventeen occurred in postoperative scars. Endocrine disturbances have aIso been considered as a cause. Lipschutz2 and his coworkers using estrogenic substances have produced fibromyomas in the abdomina1 waIIs of guinea pigs. These experimentaIIy produced tumors regressed in size when administration of estrogen was discontinued. Geschickter and Lewis,6 in examining a desmoid tumor of the abdomina1 waI1, found that it contained 13,000 rat units of gonadotropic substance per kiIogram of tissue. After studying severa connective tissue tumors, these authors concIuded that endocrine substances may play a part in their etioIogy.
to infiItrate muscle and adhere to adjacent bone. Over many years they attain great size. The Iargest reported weighed 17 kg.7 Montgomery and BIand8 excised one from the abdomina1 waI1 weighing 8.6 kg. OccasionaIIy maIignant transformation to a Iow-grade fibrosarcoma occurs, especiaIIy after numerous recurrences, but they do not metastasize. HistoIogicaIIy, they resembIe fibromas containing a varying degree of ceIIuIarity. CharacteristicaIIy a desmoid enguIfs striated muscIe fibers producing their gradua1 degeneration. The pectoraIis major, sternocIeidomastoid, diagastric, masseter, biceps, extensor carpi ulnaris, rectus femoris, gIutei, Ievator ani,g hamstring, muscIes of the dorsum of the foot, . and scapuIar region have been invoIved.3 DIAGNOSIS
The presence of a firm, fixed, non-tender, soIitary, uniIatera1, round or ova1 growth accompanied with pain is suggestive of a desmoid tumor. Musgrove and McDonaId2 state that fibrosarcoma, therapeuticaIIy the most important differentia1 diagnosis, differs from a desmoid in that “ (I) it tends to be Iarger, (2) is usuaIIy round, (3) softer in consistency, (4) in more than 50 per cent of cases it is encapsuIated, and in the remainder, is we11 circumscribed, (5) metastasis is common, and (6) the surface usuaIIy has a saImon pink homogeneous
PATHOLOGY
Desmoid tumors have been defined as nonencapsuIated, fibrous, neopIasms of tendonIike consistency arising from fascia and musstructures. These tumors cuIo-aponeurotic aIthough benign have invasive quaIities. They appear as soIitary, uniIatera1, firm, usuaIIy ovaI, sIowIy growing masses with a tendency 286
Desmoid
Tumor
of ScapuIar
Region
Two were cured, one controIIed but Iater Iost to foIIow-up. Of twenty-six cases of extra-abdomina1 desmoid tumors foIIowed one year or Ionger, twenty (77 per cent) recurred one or more times without evidence of sarcomatous change. Recurrences usuaIIy appeared during the first six months and aIways within two years aft& treatment.
appearance.” HistoIogicaIIy, the diagnosis of maIignancy may be based on the presence of pathoIogic mitotic figures, tumor giant ceIIs or encapsuIation and a high degree of ceIIuIarity. TREATMENT
The majority of writers advise wide radica1 excision as the treatment of choice. This is emphasized because of the highIy invasive quaIity of the tumor. Roentgen therapy is of IittIe, if any, additiona vaIue, aIthough Ewing stated that most of these tumors respond sIowIy but satisfactoriIy to irradiation. Roentgen therapy shouId be reserved for recurrences, the onIy partiaIIy resectabIe case, and for patients who refuse operation or are poor operative risks. High voItage roentgens or radium eIement pack therapy of over 5,000 roentgens are required. Pack and EhrIich’ suggested that the response of abdomina1 waI1 desmoids to irradiation may resuIt from suppression or cessation of ovarian function. They found that 1,000 to 1,500 roentgens directed to the tumor did not retard growth, but that at Ieast 5,000 roentgens with the porta incIuding the entire Iower abdomen are required to produce satisfactory contro1 of the tumor. In addition to radiation castration hormone therapy consisting of Iarge dosages of antifibromatogenic steroIs as testosterone, progesterone and desoxycorticosterone may prove of value in the onIy partiaIIy resectabIe cases.
SCMMARY
AND
CONCLUSIONS
A case of extra-abdomina1 desmoid tumor of the scapuIar region treated by radica1 excision and partia1 scapuIectomy with recurrence is reported. Reports of these tumors are extremeIy rare, but one seeing a firm, fixed, solitary, sIowIy growing tumor accompanied with duI1 pain shouId consider a desmoid tumor. Treatment recommended by most authorities is wide IocaI excision. Recurrences are frequent (77 per cent).2 These usuaIIy appear within the first six months and a11 within the first two years foIIowing treatment. REFERENCES I. PACK, G. T.
and EHRLICH, H. E. NeopIasms of anterior abdomina1 wall with special consideration of desmoid tumors; experience with 391 cases and coIIective review of literature. Surg., Gynec. & Obst., 79: 177-194, 1944. 2. MUSGROVE, J. E. and MCDONALD, J. R. Extraabdomina1 desmoid tumors; their differentia1 diagnosis and treatment. Arch. Patb., 45: 513540, 1948. 3. WIPER, T. B. and MILLER, J. M. Desmoid of anterior chest waI1. Am. J. Surg., 71: 556-557, 1946. 4. PENICK, R. M. Desmoid tumors deveIopine: in operative scars. Internet. S. Digest, 23: 325-329,
PROGNOSIS
WhiIe desmoid tumors are considered benign and without metastasis, IocaI recurrences are common and maIignant change occasiona1. Of 107 desmoid tumors foIIowed by Pfeiffe9 thirty-three (30.8 per cent) recurred. Eighteen recurred among eighty-five femaIes (21.2 per cent) and fifteen in twenty-two maIe patients (68.1 per cent). Pack and EhrIich’ cited eight recurrences in seventeen patients with desmoid tumors of the anterior abdomina1 waI1. They also found maIignant change more common in maIes and without reIation to size or duration of the tumor. Of seventeen cases tabuIated, three (one femaIe and two maIes) underwent maIignant change foIIowing two to six recurrences.
‘937. 5. GESCHICKTER, C. F. and LEWIS, D. Tumors of connective tissue. Am. J. Cancer, 25: 630-655, 1935. 6. EWING, J. NeopIastic Disease, 4th ed. PhiIadeIphia, 1941. W. B. Saunders. 7. STEWART, M. J. and MOUAT, T. B. Fibroma of abdomina1 waI1. Brit. J. Surg., 12: 355-377,1g24. 8. MONTGOMERY, E. E. and BLAND, P. B. Large desmoid tumor of the abdomina1 waI1. Am. Med. Pbila.. IO: 463, 1904. g. B~u~sc&wrc,~ A-.. Excision of Iarge desmoid tumor of pelvic outIet necessitating obIation of right Ievator ani muscIe. New York State J. Med. 50: 1005-1006, 1950. IO. PFEIFFER. C. Die Desmoide der Bauchdecken und ihre Prognose, Beitr. z. klin Cbir., 44: 334-901, 1904.
287