Journal of Surgical Oncology 49:113-115 (1992)
Experience With Desmoid Tumor in a Community Teaching Hospital RICHARD E. WELLING, MD, MARK E. HERMANN, BS, AND GREGORY C. KASPER, BS From the Departments of Surgery (R.E.W.) and Surgical Research (M.E.H., G.C.K.), Good Samaritan Hospital, Cincinnati, Ohio
Desmoid tumors are rare fibroblastic proliferations that occur primarily in the abdomen and extremities. They have an estimated incidence in the United States of two to four per million inhabitants per year. A recent case of desmoid tumor prompted us to review past medical records for previous cases with the same diagnosis. Since 1985, our institution has treated five patients with a total of eight desmoid tumors. Four patients (80%) were female with ages ranging from 24 to 32 years. The other patient was a 22-year-old male with a history of Gardner’s syndrome and recurrent desmoid tumors. The majority of our patients were females in the fertile age group, substantiating the hormonal effects believed to play a role in the development of these lesions. In addition, it became obvious that more investigations must be done in appropriate treatment of desmoid tumors and the documentation of these tumors in registries would be of significant benefit.
KEYWORDS:fibromatoses, musculoaponeurotic, tumor INTRODUCTION Desmoid tumors are a type of fibromatoses that are usually seen in deep musculoaponeurotic structures, primarily of the trunk and extremities. They are classified separately from benign fibrous lesions because of their infiltrative growth patterns and high rates of recurrence. Yet they are not classified as fibrosarcomas because they do not metastasize. Desmoid tumors are also further categorized by their location: either extra-abdominal, abdominal wall, or intra-abdominal [I]. This report describes our experience with five patients presenting with abdominal wall, intra-abdominal, or neck desmoid tumors and outlines the current method used in treating these lesions.
CASE REPORTS Patient 1 A 24-year-old female presented on 3/6/85 with a mass of 3 months’ duration in the right upper quadrant of her abdomen. Surgical resection of the tumor and surrounding fascia of the rectus abdominis muscle was undertaken with wide margins. Pathological inspection showed some tumor extending close to the surgical margin and verified 0 1992 Wiley-Liss, Inc.
the specimen as desmoid tumor. She did not receive adjuvant treatment.There has been no recurrence.
Patient 2 A 3 1-year-old female presented on 5/7/85 with a mass of one month’s duration on her anterior abdominal wall. The tumor was excised along with fascia of the rectus muscle. Pathological inspection verified the diagnosis of desmoid tumor but did not comment on the surgical margins. At 5-year follow-up the patient reported no recurrences. Patient 3 A 22-year-old male presented on 3/28/90 for excision of a mass in his pelvis. He had also undergone three operations since 1986 for excision of three abdominal wall desmoids. He has a history of Gardner’s syndrome and is status-post a total colectomy for familial polyposis. Excision was difficult due to the tumor’s involvement with the bladder and the ileal pouch created during his total colectomy . Pathological inspection verified the diAccepted for publication November 12, 1991. Address reprint requests to Richard E. Welling, MD, Department of Medical Research, Good Samaritan Hospital, 3217 Clifton Avenue, Cincinnati, OH 45220-2489.
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agnosis of desmoid tumor but was uncertain of its involvement with the surgical margins. He has had no recurrence.
Patient 4 A 32-year-old female presented on 5/1/90 for removal of a pelvic mass. The patient had attempted vaginal delivery 6 weeks earlier but required a caesarean-section due to the presence of the pelvic mass. The mass was removed with a transacral approach. The tumor was adherent to the coccyx and part of this was removed with the tumor. Pathological inspection verified the diagnosis of desmoid tumor with negative surgical margins. At 15 months, the patient reported no recurrences. Patient 5 A 24-year-old female presented on 2/15/91 with a 1.5 cm anterior cervical mass and a previous history of Hodgkin’s disease. Excisional biopsy exhibited a fibrous mass involved with sternomastoid. The patient subsequently underwent excision of the mass by means of a modified radical neck disection on 4/8/91. The fibroma extended to posterior and medial margins of resection and partially surrounded the jugular vein but did not infiltrate it. Pathological inspection verified diagnosis of desmoid tumor. The patient has since completed 8 weeks of radiation therapy and reports no recurrences.
DISCUSSION Desmoid tumors are extremely rare lesions with an estimated incidence in the United States of two to four per million inhabitants per year. Reitamo et al. [2] discovered that the occurrence of desmoid tumors is related to the age of the patient. They identified four age groups, each demonstrating different varieties of desmoid tumors. Fertile women in the age range of 22.8-31.6 years old displayed the highest frequency of desmoids. In this group, abdominal tumors were very prevalent. In addition, Reitamo et al. [2] speculated that the growth rates of the tumors roughly correlated with the estrogen levels of the patients. Growth was slowest in young girls, increasing from menarche and declining after menopause. The growth rate was constant in male patients throughout all the various ages. We feel our experience with desmoid tumors substantiates this observation. Two of our patients were in the fertile age group and presented with abdominal wall masses. One other patient presented with a less common, large pelvic desmoid tumor that interfered with the delivery of her child, and a fourth presented with a right neck desmoid mass. Our results, along with reports in the literature, show that desmoid tumors can be a significant problem for women of this age group, especially during pregnancy [3-61.
Our final case illustrates the problems desmoid tumors can create for patients diagnosed with Gardner’s syndrome. This autosomal-dominant defect includes soft tissue masses, bony tumors and polyposis coli [7]. Desmoid tumors are extremely common among patients with Gardner’s syndrome. In addition, these lesions have a strong, aggressive, invasive growth pattern with a high incidence of recurrence [7]. In patients with Gardner’s syndrome, desmoids seem to appear after colectomy for polyposis coli [ 1-71. Again, our experience substantiates these findings. Patient 3 first underwent a total colectomy with ileal pouch and ileal anal anastomosis 1.5 years before the appearance of his first desmoid tumor. Since then, he has had two recurrent abdominal wall tumors as well as his most recent pelvic mass. The treatment of choice for desmoid tumors is total excision [8,9]. Despite excision with margins free of disease, recurrence rates still may be as high as 40% [9]. Radiation therapy has been suggested as adjunct treatment or as primary therapy for desmoid tumors that would require mutilating surgery. However, these studies were performed on extra-abdominal desmoids [9]. Chemotherapy has also been suggested for these tumors Pl101.
Desmoid tumors can usually be surgically treated in a definitive manner. Still, when the tumor recurs or cannot be excised completely, adjuvant therapy may be necessary. Unfortunately, long-term outcome under these conditions is not available due to the rarity of these tumors. Therefore, we concur with Lopez et al. that these tumors should be treated as low-grade malignancies and followed by tumor registries [ 111.
REFERENCES 1. Enzinger FM, Weiss SW (eds): “Soft Tissue Tumors.” St. Louis:
CV Mosby, 1983. 2. Reitamo JJ, Scheinin TM, Hayry P: The desmoid syndrome: New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg 151:230-237, 1986. 3. Khoo SK: The desmoid tumor, a diagnostic problem in the gynecological patient: A report of four patients. Aust NZ J Surg 42:17&-174, 1972. 4. Buckshee K, Mittal S, Agarwal N, et al.: Pelvic fibromatoses: A rare gynecological entity. Int J Gynecol Obstet 26:459-361, 1988. 5. Simon NL, Mazur MT, Shingleton HM: Pelvic fibromatosis: An unusual gynecologic tumor. Obstet Gynecol65:767-769, 1985. 6. Carnie1 MR, Solish GI: Desmoid tumor during pregnancy. Am J Obstet Gynecol 144:988-989, 1982. 7. Zissiadis A, Harlaftis N, Aletras H: Desmoid tumor in Gardner’s syndrome. Am Surg 56:305-307, 1990. 8. Miralbell R, Suit HD, Mankin HJ, et al.: Fibromatoses: From postsurgical surveillance to combined surgery and radiation therapy. Int J Radiat Oncol Biol Phys 18535-540, 1990. 9. Easter DW, Halasz NA: Recent trends in the management of desmoid tumors: Summary of 19 cases and review of the literature. Ann Surg 210:765-769, 1989. 10. Waddell WR, Gerner RE: Indomethacin and ascorbate inhibit desmoid tumors. J Surg Oncol 1535-90, 1980.
Desmoid Tumor 1 1 , Lopez R, Kemalyan N, Moseley HS, et al.: Problems in diagnosis and management of desmoid tumors. Am J Surg 159:45W53, 1990.
EDITORIAL COMMENTS Desmoid tumors are classified as benign tumors in some textbooks because they never metastasize. On the other hand, they differ from some other types of fibromatoses because they are locally invasive and if located in strategic anatomical areas such as the base of the mesentery, may ultimately cause the demise of the patient. The authors’ admonition that desmoid tumors should be considered as low-grade malignancies is widely accepted by surgical oncologists. Although there are reports in the literature indicating occasional regression of these tumors with anti-estrogen or anti-inflammatory therapy [ 1-41 and long-term stabilization or regression with radiation therapy, wide surgical resection remains the primary modality of treatment. In our experience at Roswell Park Cancer Institute wide resection or conservative resection followed by adjuvant radiation appears to control the
115
disease in the vast majority of patients with trunk or extremity desmoids. Local recurrences should be aggressively pursued. Desmoids associated with the Gardner’s syndrome constitute a specific entity which is more difficult to treat with varying therapeutic and prognostic experiences at various centers. Constantine P. Karakousis, MD, Roswell Park Cancer Institute Buffalo, NY 14263
PhD
REFERENCES 1. Kinsbrunner B, Ritter S , Domingo J, et al.: Remission of rapidly
growing desmoid tumors after tamoxifen therapy. Cancer 52:220 I2204, 1983. 2. Lanari A: Effect of progesterone on desmoid tumors (aggressive fibromatosis). N Engl J Med 309: 1523, 1983, 3. Waddell WR: Treatment of intra-abdominal and abdominal wall desmoid tumors with drugs that affect the metabolism of cyclic 3’,5’-adenosine monophosphate. Ann Surg 181 :299-302, 1975. 4. Waddell WR, Gemer RE: Indomethacin and ascorbate inhibit desmoid tumors. J Surg Oncol 15:85-90, 1980.
Experience With Desmoid Tumor in a Community Teaching Hospital RICHARD E. WELLING, MD, MARK E. HERMANN, BS, AND GREGORY C. KASPER, BS From the Departments of Surgery (R.E.W.) and Surgical Research (M.E.H., G.C.K.), Good Samaritan Hospital, Cincinnati, Ohio
Desmoid tumors are rare fibroblastic proliferations that occur primarily in the abdomen and extremities. They have an estimated incidence in the United States of two to four per million inhabitants per year. A recent case of desmoid tumor prompted us to review past medical records for previous cases with the same diagnosis. Since 1985, our institution has treated five patients with a total of eight desmoid tumors. Four patients (80%) were female with ages ranging from 24 to 32 years. The other patient was a 22-year-old male with a history of Gardner’s syndrome and recurrent desmoid tumors. The majority of our patients were females in the fertile age group, substantiating the hormonal effects believed to play a role in the development of these lesions. In addition, it became obvious that more investigations must be done in appropriate treatment of desmoid tumors and the documentation of these tumors in registries would be of significant benefit.
KEYWORDS:fibromatoses, musculoaponeurotic, tumor INTRODUCTION Desmoid tumors are a type of fibromatoses that are usually seen in deep musculoaponeurotic structures, primarily of the trunk and extremities. They are classified separately from benign fibrous lesions because of their infiltrative growth patterns and high rates of recurrence. Yet they are not classified as fibrosarcomas because they do not metastasize. Desmoid tumors are also further categorized by their location: either extra-abdominal, abdominal wall, or intra-abdominal [I]. This report describes our experience with five patients presenting with abdominal wall, intra-abdominal, or neck desmoid tumors and outlines the current method used in treating these lesions.
CASE REPORTS Patient 1 A 24-year-old female presented on 3/6/85 with a mass of 3 months’ duration in the right upper quadrant of her abdomen. Surgical resection of the tumor and surrounding fascia of the rectus abdominis muscle was undertaken with wide margins. Pathological inspection showed some tumor extending close to the surgical margin and verified 0 1992 Wiley-Liss, Inc.
the specimen as desmoid tumor. She did not receive adjuvant treatment.There has been no recurrence.
Patient 2 A 3 1-year-old female presented on 5/7/85 with a mass of one month’s duration on her anterior abdominal wall. The tumor was excised along with fascia of the rectus muscle. Pathological inspection verified the diagnosis of desmoid tumor but did not comment on the surgical margins. At 5-year follow-up the patient reported no recurrences. Patient 3 A 22-year-old male presented on 3/28/90 for excision of a mass in his pelvis. He had also undergone three operations since 1986 for excision of three abdominal wall desmoids. He has a history of Gardner’s syndrome and is status-post a total colectomy for familial polyposis. Excision was difficult due to the tumor’s involvement with the bladder and the ileal pouch created during his total colectomy . Pathological inspection verified the diAccepted for publication November 12, 1991. Address reprint requests to Richard E. Welling, MD, Department of Medical Research, Good Samaritan Hospital, 3217 Clifton Avenue, Cincinnati, OH 45220-2489.
114
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agnosis of desmoid tumor but was uncertain of its involvement with the surgical margins. He has had no recurrence.
Patient 4 A 32-year-old female presented on 5/1/90 for removal of a pelvic mass. The patient had attempted vaginal delivery 6 weeks earlier but required a caesarean-section due to the presence of the pelvic mass. The mass was removed with a transacral approach. The tumor was adherent to the coccyx and part of this was removed with the tumor. Pathological inspection verified the diagnosis of desmoid tumor with negative surgical margins. At 15 months, the patient reported no recurrences. Patient 5 A 24-year-old female presented on 2/15/91 with a 1.5 cm anterior cervical mass and a previous history of Hodgkin’s disease. Excisional biopsy exhibited a fibrous mass involved with sternomastoid. The patient subsequently underwent excision of the mass by means of a modified radical neck disection on 4/8/91. The fibroma extended to posterior and medial margins of resection and partially surrounded the jugular vein but did not infiltrate it. Pathological inspection verified diagnosis of desmoid tumor. The patient has since completed 8 weeks of radiation therapy and reports no recurrences.
DISCUSSION Desmoid tumors are extremely rare lesions with an estimated incidence in the United States of two to four per million inhabitants per year. Reitamo et al. [2] discovered that the occurrence of desmoid tumors is related to the age of the patient. They identified four age groups, each demonstrating different varieties of desmoid tumors. Fertile women in the age range of 22.8-31.6 years old displayed the highest frequency of desmoids. In this group, abdominal tumors were very prevalent. In addition, Reitamo et al. [2] speculated that the growth rates of the tumors roughly correlated with the estrogen levels of the patients. Growth was slowest in young girls, increasing from menarche and declining after menopause. The growth rate was constant in male patients throughout all the various ages. We feel our experience with desmoid tumors substantiates this observation. Two of our patients were in the fertile age group and presented with abdominal wall masses. One other patient presented with a less common, large pelvic desmoid tumor that interfered with the delivery of her child, and a fourth presented with a right neck desmoid mass. Our results, along with reports in the literature, show that desmoid tumors can be a significant problem for women of this age group, especially during pregnancy [3-61.
Our final case illustrates the problems desmoid tumors can create for patients diagnosed with Gardner’s syndrome. This autosomal-dominant defect includes soft tissue masses, bony tumors and polyposis coli [7]. Desmoid tumors are extremely common among patients with Gardner’s syndrome. In addition, these lesions have a strong, aggressive, invasive growth pattern with a high incidence of recurrence [7]. In patients with Gardner’s syndrome, desmoids seem to appear after colectomy for polyposis coli [ 1-71. Again, our experience substantiates these findings. Patient 3 first underwent a total colectomy with ileal pouch and ileal anal anastomosis 1.5 years before the appearance of his first desmoid tumor. Since then, he has had two recurrent abdominal wall tumors as well as his most recent pelvic mass. The treatment of choice for desmoid tumors is total excision [8,9]. Despite excision with margins free of disease, recurrence rates still may be as high as 40% [9]. Radiation therapy has been suggested as adjunct treatment or as primary therapy for desmoid tumors that would require mutilating surgery. However, these studies were performed on extra-abdominal desmoids [9]. Chemotherapy has also been suggested for these tumors Pl101.
Desmoid tumors can usually be surgically treated in a definitive manner. Still, when the tumor recurs or cannot be excised completely, adjuvant therapy may be necessary. Unfortunately, long-term outcome under these conditions is not available due to the rarity of these tumors. Therefore, we concur with Lopez et al. that these tumors should be treated as low-grade malignancies and followed by tumor registries [ 111.
REFERENCES 1. Enzinger FM, Weiss SW (eds): “Soft Tissue Tumors.” St. Louis:
CV Mosby, 1983. 2. Reitamo JJ, Scheinin TM, Hayry P: The desmoid syndrome: New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg 151:230-237, 1986. 3. Khoo SK: The desmoid tumor, a diagnostic problem in the gynecological patient: A report of four patients. Aust NZ J Surg 42:17&-174, 1972. 4. Buckshee K, Mittal S, Agarwal N, et al.: Pelvic fibromatoses: A rare gynecological entity. Int J Gynecol Obstet 26:459-361, 1988. 5. Simon NL, Mazur MT, Shingleton HM: Pelvic fibromatosis: An unusual gynecologic tumor. Obstet Gynecol65:767-769, 1985. 6. Carnie1 MR, Solish GI: Desmoid tumor during pregnancy. Am J Obstet Gynecol 144:988-989, 1982. 7. Zissiadis A, Harlaftis N, Aletras H: Desmoid tumor in Gardner’s syndrome. Am Surg 56:305-307, 1990. 8. Miralbell R, Suit HD, Mankin HJ, et al.: Fibromatoses: From postsurgical surveillance to combined surgery and radiation therapy. Int J Radiat Oncol Biol Phys 18535-540, 1990. 9. Easter DW, Halasz NA: Recent trends in the management of desmoid tumors: Summary of 19 cases and review of the literature. Ann Surg 210:765-769, 1989. 10. Waddell WR, Gerner RE: Indomethacin and ascorbate inhibit desmoid tumors. J Surg Oncol 1535-90, 1980.
Desmoid Tumor 1 1 , Lopez R, Kemalyan N, Moseley HS, et al.: Problems in diagnosis and management of desmoid tumors. Am J Surg 159:45W53, 1990.
EDITORIAL COMMENTS Desmoid tumors are classified as benign tumors in some textbooks because they never metastasize. On the other hand, they differ from some other types of fibromatoses because they are locally invasive and if located in strategic anatomical areas such as the base of the mesentery, may ultimately cause the demise of the patient. The authors’ admonition that desmoid tumors should be considered as low-grade malignancies is widely accepted by surgical oncologists. Although there are reports in the literature indicating occasional regression of these tumors with anti-estrogen or anti-inflammatory therapy [ 1-41 and long-term stabilization or regression with radiation therapy, wide surgical resection remains the primary modality of treatment. In our experience at Roswell Park Cancer Institute wide resection or conservative resection followed by adjuvant radiation appears to control the
115
disease in the vast majority of patients with trunk or extremity desmoids. Local recurrences should be aggressively pursued. Desmoids associated with the Gardner’s syndrome constitute a specific entity which is more difficult to treat with varying therapeutic and prognostic experiences at various centers. Constantine P. Karakousis, MD, Roswell Park Cancer Institute Buffalo, NY 14263
PhD
REFERENCES 1. Kinsbrunner B, Ritter S , Domingo J, et al.: Remission of rapidly
growing desmoid tumors after tamoxifen therapy. Cancer 52:220 I2204, 1983. 2. Lanari A: Effect of progesterone on desmoid tumors (aggressive fibromatosis). N Engl J Med 309: 1523, 1983, 3. Waddell WR: Treatment of intra-abdominal and abdominal wall desmoid tumors with drugs that affect the metabolism of cyclic 3’,5’-adenosine monophosphate. Ann Surg 181 :299-302, 1975. 4. Waddell WR, Gemer RE: Indomethacin and ascorbate inhibit desmoid tumors. J Surg Oncol 15:85-90, 1980.
