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Journal of Clinical Oncology, Vol 26, No 15 (May 20), 2008: pp. 2586-2589 © 2008 American Society of Clinical Oncology. DOI: 10.1200/JCO.2007.15.6034
Management of a Presacral Teratoma in an AdultSection of Surgical Oncology, Department of Surgery, Boston University School of Medicine, Boston, MA
Department of Obstetrics and Gynecology, Boston University School of Medicine, Boston, MA A 26-year-old female presented to a free-standing family clinic in August 2007 for an elective termination of pregnancy. Initial ultrasound confirmed a viable 9-week intrauterine pregnancy. On bimanual examination a 15-cm fixed pelvic mass was palpated, and a mass effect from the left adnexal region, shifting the entire vaginal wall to the right, was observed with speculum examination. Attempted termination of pregnancy via the vaginal route was precluded as displaced tissue extended into the introitus and left rectal fossa impeded the visualization of the cervix. The patient was subsequently referred to Boston University Medical Center for further evaluation of her pelvic mass and pregnancy termination. The patient's past medical and surgical history were pertinent only for dysmenorrhea, appendectomy, and small bowel resection for Meckel's diverticulum. She denied pelvic pain, dysparenuria, or bowel or bladder symptoms. Pertinent physical findings were limited to a fullness appreciated on the left side of the vulva, shifting the vulva to the right, and a soft, nontender mass in the left hemipelvis. The patient was slightly anemic (hematocrit of 33%) and had a normal CA-125 level. Repeat ultrasonography confirmed within the mid to left pelvis, inferior to the uterus, a large, complex, solid, and cystic mass, with internal frond-like projections measuring 11 x 10 x 8 cm. No definite internal septations or vasculature were noted within the lesion. Axial computed tomography (CT) scan revealed a 10 x 9.3 cm well-defined, low-density, heterogeneous mass in the ischiorectal space (Fig 1A; arrowhead), to the left of and displacing the rectum and vaginal cuff to the right (Fig 1A; arrow). The mass also seemed to be distinct and separate from the uterus, cervix, vagina, and both ovaries (Fig 1B: sagittal CT showing the teratoma in relation to the gravid uterus, bladder, sacrum, and other surrounding structures). The low-density attenuation characteristics of the lesion suggested the possibility of a fatty component, such as a liposarcoma. Posterior to the large mass was a smaller, 2.5 x 2 cm circumscribed fat density in the subcutaneous tissue of the left ischiorectal fat pad (Fig 2: an axial CT of the second mass, posterior to the teratoma in the ischiorectal fat [arrow]). There was no evidence of adenopathy or metastatic disease within the abdomen. Magnetic resonance imaging (MRI) demonstrated that the mass seemed to be located above the levator ani with the posterior margin approximately 1 cm anterior to the coccyx. Within the mass were multiple soft tissue nodules, consistent with fatty elements; much of the lesion was composed of fluid with no gross areas of signal void suggesting calcification (Fig 3: sagittal MRI of the teratoma located approximately 1 cm anterior to the coccyx with no evidence of infiltration of the surrounding soft tissues, adenopathy, or significant free fluid). Our differential included adult paracoccygeal teratoma or liposarcoma. On August 10, 2007, the patient underwent a transabdominal pelvic exploration, bilateral ureterolysis, and resection of both pelvic masses with vaginal repair. Postoperative gross examination of the presacral tumor is seen in Fig 4. Pregnancy termination was completed by hysterotomy and suction curettage. Microscopic tissue examination of the primary mass revealed an 8.5-cm mature cystic teratoma (Fig 5: histologic section [4x, hematoxylin and eosin] of the presacral teratoma reveals a cystic mass lined by squamous epithelium, surrounded by fibrous and smooth muscle stroma containing sebaceous glands). The second, smaller ischiorectal fossa mass demonstrated a cyst with a dense fibrotic wall showing chronic inflammation, foreign material, foreign body giant cell reaction, and dystrophic calcification. The patient had no postoperative complications and was discharged home from the hospital on postoperative day 5. On her postoperative outpatient visit, she had normal anal sphincter tone with bowel and bladder continence fully intact, the vaginal repair was well-healed and she was sensory intact.
Presacral teratomas are the most frequently recognized fetal neoplasm, with a reported incidence of 1 in 35,000 to 40,000 births.1 The majority of these tumors are benign, but the incidence of malignancy ranges from 10% to 50% in infants and increases with age. In adults, they are slowly expanding, highly differentiated tumors of dermoid composition. Early and complete surgical resection is the foundation of successful therapy and results in cure rates that exceed 95%. Rarely do presacral teratomas present in adulthood. The first reported case of a presacral teratoma in an adult was published by Emmerich in 1847 and was included as one of six cases reviewed by Kiderlen in 1899.2 A review of world literature from the early 1900s to the present demonstrates fewer than 120 reported cases of isolated adult presacral teratoma.1,3-14 Of those patients whose sex was reported (92 of 104 patients), 70% were female and 30% were male. Furthermore, only four cases have been documented from the United States since 1975, two of which were found in females during pregnancy.1,15 Our case is an addition to the series. Extragonadal teratomas derive from totipotential cells of primordial germ cell or early embryonic cell origin.2 The designation, teratoma, refers to a varied group of tumors that show differentiation toward somatic-type tissues that can be typical of either adult or embryonic development. They develop from the three embryonic germ cell layers, are typified by the presence of virtually any tissue type, and are most commonly located in the sacrococcygeal (57%) region. Teratomas are categorized as mature (cystic or solid, benign), immature (malignant tissue of germ cell origin), and monodermal or highly specialized.10,15 According to the Altman Classification of the Surgical Section of the American Academy of Pediatrics, sacrococcygeal teratomas are divided into four types: type I (46%), tumors that are predominantly external, projecting from the sacrococcygeal region and presenting with distortion of the buttocks; type II (35%), tumors that are predominantly external but have a large intrapelvic component; type III (9%), those that are predominantly intrapelvic, with a small external, buttock mass; type IV (10%), those that are entirely internal with no external or buttock component.16 We define a true presacral teratoma as one that has no sacrococcygeal component and is fully independent of any clearly apparent tissue connections to the coccyx. Presacral teratomas grow posteroinferiorly into the gluteal area and/or anterosuperiorly into the abdominopelvic cavity. Symptoms specific to the sacrococcygeal and presacral tumor locations are those of mass effects displacing contiguous structures: rectal pain, backache, paresthesias, numbness in the so-called saddle area and legs, and loss of bladder and rectal control. Advanced symptoms may not indicate tumor unresectability. With our patient, it was difficult to determine the relationship of the mass to the levator muscles and whether this was in fact an intrapelvic tumor or a buttock sarcoma. Imaging such as ultrasound, CT, and MRI can be extremely helpful and complementary to diagnosis. A preoperative incisional biopsy may also be an appropriate diagnostic component if an extremity sarcoma is high on one's differential. As presacral tumors may or may not extend to the coccyx, it is important to distinguish true sacrococcygeal teratomas from independent presacral teratomas to most appropriately manage the patient. Treatment for presacral teratomas ideally includes complete surgical resection of the tumor. The surgical approach may be a complex decision. One must take into account tumor size, location, relationship to surrounding tissues, the patient's overall health, and other determinants. Surgical options include a posterior, trans-sacral Kraske approach with exposure of the presacral retrotrectal space, a technique that involves excision of the coccyx and lower sacral segments. Other potential approaches include a transabdominal approach, combined transabdominal and perineal approach, combined abdominosacral approach, or more recently, laparoscopic approaches. For benign disease, no further adjuvant therapy may be necessary. Traditional treatment of sacrococcygeal teratomas favors always removing the coccyx with the tumor, since failure to do so results in an approximately 30% to 40% recurrence rate. Yet previous case reports fail to recognize the important distinction between isolated presacral teratomas (defined here as those with no apparent tissue attachment to the coccyx) and true sacrococcygeal teratomas. Failure to differentiate between the two entities could lead to unnecessary coccygectomies and increased time spent in the operating room. Complications seen with coccygectomy are delayed wound healing with increased wound infection rate, increased recovery time, and rarely, bowel herniation.17,18 The presacral tumor in our patient had no association with the coccyx and a posterior margin of approximately 1 cm anterior to the coccyx, so we felt that complete surgical resection of the tumor alone was adequate treatment. Because intraoperative histologic evaluation of the tumor demonstrated a mature, benign cystic teratoma, our patient needed no further therapy. For malignant disease, a more aggressive, multimodal approach with possible combination of chemotherapy, radiation therapy, and surgery may be indicated.19,20 No concrete standards for therapy exist and multiple protocols have been used in the past. Additionally, there are no clinical trials to date to test the most optimal treatments, thus the best option is to refer the patient to an advanced oncology center. AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest.
ACKNOWLEDGMENTS We thank Jianmin Gan, MD, and the Department of Pathology at Boston University Medical Center for their participation in the histologic sectioning and staining of the tumor and for providing us with the images that we used in this case report. REFERENCES 1. Ng EW, Porcu P, Loehrer PJ Sr: Sacrococcygeal teratoma in adults: Case reports and a review of the literature. Cancer 86:1198-1202, 1999[CrossRef][Medline] 2. Head HD, Gerstein JD, Muir RW: Presacral teratoma in the adult. Am Surg 41:240-248, 1975[Medline] 3. Hiroyuki A, Morihito M, Toshiaki U, et al: A case of adult sacrococcygeal teratoma usefulness of helical computed tomographic examination in decision-making on operation method and position. J Japan Soc Colo-Proctol 54:267-272, 2001 4. Chene G, Voitellier M: Benign pre-sacral teratoma and vestigial retrorectal cysts in the adult [in French]. J Chir (Paris) 143:310-314, 2006[Medline] 5. Ha MT, Cho IM, Joung SY, et al: A case of presacral teratoma in an adult female. Korean J Obstet Gynecol 49:2631-2635, 2006 6. Juca M, de Oliveira FF, Gomes EG, et al: Sacrococcycygeal teratoma in adult: Report of a case. Int J Gastrointest Cancer 37:91-93, 2006[Medline] 7. Li Y, Jiang Y, Song S: Diagnosis and treatment of primary retroperitoneal teratoma [in Chinese]. Zhonghua Wai Ke Za Zhi 38:892-894, 2000[Medline] 8. Luong TV, Salvagni S, Bordi C: Presacral carcinoid tumour. Review of the literature and report of a clinically malignant case. Dig Liver Dis 37:278-281, 2005[CrossRef][Medline] 9. Matsumura Y, Okumura K, Imamura M, et al: Adult sacrococcygeal teratoma: A case report [in Japanese]. Hinyokika Kiyo 48:599-601, 2002[Medline] 10. Monteiro M, Cunha TM, Catarino A, et al: Case report: Sacrococcygeal teratoma with malignant transformation in an adult female—CT and MRI findings. Br J Radiol 75:620-623, 2002 11. Noguera R, Navarro S, Carda C, et al: Near-haploidy in a malignant sacrococcygeal teratoma. Cancer Genet Cytogenet 108:70-74, 1999[CrossRef][Medline] 12. Saygun O, Avsar FM, Topaloglu S, et al: Retrorectal benign cystic teratoma misdiagnosed with high lying pararectal abscess. Hepatogastroenterology 50:cclii-ccliii, 2003 (suppl 2)[Medline] 13. Shin B, Bae E, Kang GJ, et al: Two cases of presacral teratomas in adult. Korean J Obstet Gynecol 43:2050-2054, 2000 14. Takahashi K, Shinno T, Watanabe Y, et al: Benign teratoma in an 85-year-old woman. Arch Gynecol Obstet 263:188-190, 2000[CrossRef][Medline] 15. Bull J Jr, Yeh KA, McDonnell D, et al: Mature presacral teratoma in an adult male: A case report. Am Surg 65:586-591, 1999[Medline] 16. Altman RP, Randolph JG, Lilly JR: Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey-1973. J Pediatr Surg 9:389-398, 1974[Medline] 17. Schropp KP, Lobe TE, Rao B, et al: Sacrococcygeal teratoma: The experience of four decades. J Pediatr Surg 27:1075-1078, 1992[Medline] 18. Wood KB, Mehbod AA: Operative treatment for coccygodynia. J Spinal Disord Tech 17:511-515, 2004[CrossRef][Medline] 19. Biskup W, Calaminus G, Schneider DT, et al: Teratoma with malignant transformation: Experiences of the cooperative GPOH protocols MAKEI 83/86/89/96. Klin Padiatr 218:303-308, 2006[CrossRef][Medline] 20. Schneider DT, Wessalowski R, Calaminus G, et al: Treatment of recurrent malignant sacrococcygeal germ cell tumors: Analysis of 22 patients registered in the German protocols MAKEI 83/86, 89, and 96. J Clin Oncol 19:1951-1960, 2001
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Copyright © 2008 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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