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IOSR Journal of Dental and Medical Sciences (IOSR-JDMS)
e-ISSN: 2279-0853, p-ISSN: 2279-0861.Volume 14, Issue 11 Ver. IV (Nov. 2015), PP 74-76
www.iosrjournals.org
DOI: 10.9790/0853-141147476 www.iosrjournals.org 74 | Page
Benign Retroperitoneal Teratoma in young adult--A case report
and literature review
Dr.Abhishek Kr Singh1
,Dr.H.I Pandey2
, Dr.Abhay Kumar3
Department of Surgery, Tata Main Hospital, Jamshedpur
Abstract: Teratomas are the germ cell tumours which comprises of tissues from all the three germ cell layers.
Primary retroperitoneal teratoma is a relatively rare tumour in adults. The primary retroperitoneal teratoma
constitutes 6–11% of the retroperitoneal tumours. Sixty percent of the retroperitoneal teratomas occur in
children less than 15 years. It occurs more commonly in females than males. The order of frequency for
teratoma is ovary, testis, mediastinum and the retroperitoneum at last. Early diagnosis and surgery is the
mainstay of treatment. We report a rare case of retroperitoneal teratoma presented to us as left parietal wall
abscess. Exploratory laparotomy with tumour resection was performed. Histopathological diagnosis confirmed
a benign cystic teratoma. The patient is doing well and is on regular follow up.
Keywords: Retroperitoneum, teratoma, laparotomy, mediastinum
I. Introduction
Teratomas are uncommon neoplasm‟s that contain derivatives of all three germ layers (ectoderm,
mesoderm and endoderm). Historically, teratomas were attributed to demons, sexual misconduct and abnormal
fertilization. As with teratogenic, the name derives from the Greek word teras, meaning „monster‟. Teratomas
belong to a class of tumours known as non-seminomatous germ cell tumour and are typically located in gonadal
region. However, extragonadal sites such as the sacrococcygeal region, mediastinum, neck, diancephalon and
retroperitoneum have also been reported. Retroperitoneal teratomas often occur in infancy and childhood but are
rare in adults. In this article, we describe an unusual case of a retroperitoneal teratoma in a 18-year old male
patient who presented as left parietal wall abscess subsequently, underwent successful surgical resection.
II. Case Report
A 18-year old boy presented with fever on and off for last 1 month and left upper abdominal distension
for 15 days. He had no weight loss, bowel or urinary complaints. Mass was aspirated at local hospital and pus
like thing came out and later referred here. On examination, a large diffuse mass was palpable in the left flank
extending to left of umbilicus. X-ray Abdomen shows dilated bowel loop with calcification. Ultrasound
Abdomen shows large cystic lesion of 7x10cm (approx.Vol. 400 cc) with layering of its content is noted in
epigastric region. Non Contrast CT abdomen reveal few dilated bowel loops with few bowel entrapment with
target sign positive, descending bowel more dilated. Chest X-ray revealed no lung metastases or
lymphadenopathy.
Laboratory blood investigations shows leucocytosis W.B.C 14,500 and Hb-10.5gm%.Others
parameters were within normal limit. Tumour marker AFP, B-HCG and LDH were within normal values.
Fig.1 X-ray Abdomen shows calcification with dilated bowel Fig 2.NCCT scan showing mass with
the presence of calcification.
Benign Retroperitoneal Teratoma in young adult--A case report and literature review
DOI: 10.9790/0853-141147476 www.iosrjournals.org 75 | Page
In view of above findings he was taken for Emergency exploratory laparotomy.Operative finding
revealed large retroperitoneal dermoid tumour (?teratoma mature) with tooth (multiple)with hair and vascular
pedicle, tumour and pus like collection of 700 ml.Tumour was anterior to kidney, adherent in retroperitoneal
area including mesenteric vessel and adherent to aorta and renal vein and artery in close association with
pancreas and portal vein,ureter separate, mesocolon involved but vessels spared. Tumour contains pus and
sebum, hair and tooth with capsule. Macroscopically growth measured 8x6x4cm.Microscopically, section show
squamous epithelium with intact basement membrane, underneath hair follicle and osteoid material present with
areas of haemorrhage and focal chronic inflammatory exudates, few sebaceous glands. Histopathology
confirmed the diagnosis of a benign cystic teratoma, benign in nature, with no malignant cells present.
Postoperative course was uneventful. After 1 month, in follow up,he is fine with no recurrence or any
symptoms.
Fig 3.Gross appearance of retroperitoneal mass Fig.4 Microscopic examination of the tumor showing
Squamous epithelium, pilosebaceous glands
III. Discussion
Overall, primary retroperitoneal teratomas constitute about 1-11% [1-3] of all primary retroperitoneal
tumours. Approximately half of all teratomas found in children are discovered in first decade of life with 43-
45% of retroperitoneal teratomas diagnosed within the first year of life. Less than 10-20% of retroperitoneal
teratomas present in patients after 30 years of age [4].
Teratomas arise from germ cells that fail to mature normally in the gonadal locations. These totipotent
cells can differentiate into tissue components representing derivatives of mesoderm, ectoderm and endoderm.
The distribution of teratomas listed in order of decreasing frequency is: ovaries, testes, anterior mediastinum,
retroperitoneal space, pre sacral and coccygeal areas, pineal and other intracranial sites, neck and abdominal
viscera other than gonads. The developmental migratory properties of germ cells would explain teratomas in
these extragonadal sites, which generally occur along midline structures [1, 5]. Retroperitoneal teratomas are
usually asymptomatic except when compression of the surrounding structures occurs. Patients with compressive
symptoms may present with back pain, genitourinary symptoms, gastrointestinal symptoms (abdominal
distension, pain, nausea and vomiting), as well as lower extremity or genital enema secondary to lymphatic
obstruction [1, 6]. The differential diagnosis of retroperitoneal teratomas include ovarian tumors, renal cysts,
adrenal tumours, retroperitoneal fibromas, retroperitoneal sarcomas (usually liposarcomas), hemangiomas,
xanthogranulomas, enlarged lymph nodes and perirenal abscesses [7, 8].
Testicular ultrasound is necessary to rule out testicular germ cell tumour in male patients. This is a
necessary step since 50% of men with retroperitoneal tumours also have testicular carcinoma in situ, a precursor
for testicular germ cell tumours [9].
Calcifications in teratomas seen within the tumour or on the rim of the cyst wall. Even though 74% of
benign teratomas contain calcification, they also occur in 12.5% of malignant teratomas [10].
Ultrasound can identify the cystic, solid or complex components of the tumour [11]. The cystic portion
may be further differentiated into sebum, non-fat fluid and structures resembling fetal parts. However,
ultrasound has its limitations as Davidson et al found that ultrasound poorly identified fat and calcifications,
which are suggestive of teratoma.
CT gives more specific information on the fat, proteinaceous fluid and calcification components of the
teratoma. The presence of fatty portions of the tumour in the horizontal interface with dependent fluid, which
probably represents sebum, is virtually pathognomonic of a teratoma [11, 12]. However, a fat fluid level has also
Benign Retroperitoneal Teratoma in young adult--A case report and literature review
DOI: 10.9790/0853-141147476 www.iosrjournals.org 76 | Page
been described in case of a well differentiated liposarcoma of the retroperitoneum [13]. Second, CT appears
superior to ultrasound at defining extent in to surrounding organs and for evaluating the cyst wall [11].
MRI is superior to both ultrasound and CT in defining the anatomical relationship of the teratoma with
adjacent organs and local tumour spread [14, 15]. MRI can also distinguish fluid, fat, calcium and soft tissue
elements, as well as predict resectability and evaluate recurrence [16].
Angiography is beneficial for detecting the presence of hypervascularity, arterial encasement and organ
invasion, features often suggesting malignancy [17].
Surgical excision of benign (mature) teratoma is required for a definitive diagnosis (by
histopathological examination) [18] and remains the mainstay of treatment [19]. Prognosis is fortunately
excellent after complete surgical excision with an overall five-year survival rate of nearly 100% [20]. Teratomas
are largely resistant to radio- and chemotherapy. Adjuvant radio- and chemotherapy are used only if malignant
features of germ cell tumours are identified on histopathological examination [21].
IV. Conclusion
Teratomas can macroscopically be divided into 2 categories: cystic or solid. Cystic teratomas are
mostly benign, containing sebaceous materials and mature tissue types. On the other hand, solid teratomas are
often malignant and composed of immature embryonic tissues in addition to adipose, cartilaginous, fibrous and
bony components. Since it does not respond to chemotherapy and radiotherapy, surgery is the mainstay of
treatment. The prognosis is excellent for benign retroperitoneal teratoma if complete resection can be
accomplished. Close follow-up is required, as histological mature teratoma may turn malignant.
References
[1] Gschwend J, Burke TW, Woodward JE, et al. Retroperitoneal teratoma presenting as an abdominal-pelvic mass. Obstet Gynecol
1987; 70: 500-502.
[2] Lane RH, Stephens DH, Reiman HM. Primary retroperitoneal neoplasms: CT findings in 90 cases with clinical and pathologic
correlation. Amer Jour Roentgenology 1989; 152: 83-89.
[3] Wang RM, Chen CA. Primary retroperitoneal teratoma. Acta Obstet Gynecol Scand 2000; 79: 707-708.
[4] Panageas E. General diagnosis case of the day. Primary retroperitoneal teratoma. AJR Am J Roentology 1991; 156; 1292-1294
[5] Engel RM, Elkins RC, Fletcher BD. Retroperitoneal teratoma. Review of the literature and presentation of an unusual case. Cancer
1968; 22: 1068-1073.
[6] Lambrianides AL, Walker MM, Rosin RD. Primary retroperitoneal teratoma in adults. Urology 1987; 29: 310-312.
[7] Pantoja E, Llobet R, Gonzalez-Flores B. Retroperitoneal teratoma: a historical review. J Urol 1976; 115: 520-523.
[8] Pandya JS, Pai MV, Munchhala S. Retroperitoneal teratoma presenting as acute abdomen in an elderly person. Ind Jou Gastroentero
2000; 19; 89-90.
[9] T. Gilligan, P.Kantoff. Extragonadal germ cell tumors involving the mediastinum and retroperitoneum. UpToDate Patient Preview,
W.K. Oh, Ed., UpToDate, Waltham, Mass, USA, 2009.
[10] Bruneton JN, Diard F, Drouillard JP, et al. Primary retroperitoneal teratoma in adults: Presentation of two cases and review of the
literature. Radiology 1980; 134; 613-616.
[11] Davidson AJ, Hartman DS, Goldman SM, Mature teratoma of the retroperitoneum: radiologic, pathologic, and clinical correlation.
Radiology 1989; 172: 421-425
[12] Billmire DF, Grosfeld JL. Teratomas in childhood: analysis of 142 cases. J Pediatric Surg 1986; 21: 548-551.
[13] Smirniotopoulos JG, Chiechi MV. Teratomas, dermoids, and epidermoids of the head and neck. Radiographics 1995; 15: 1437-
1455.
[14] Bellin MF, Duron JJ, Curet PH, Dion-Voirin E, Grellet J. Primary retroperitoneal teratoma in the adult: correlation of the MRI
features with CT and pathology. Magn Reson Imaging 1991; 9: 263-266.
[15] Choi BI, Chi JG, kim SH, Chang KH, Han MC. MR imaging of retroperitoneal teratoma: correlation with CT and pathology. J
Comput Assist Tomogr 1989; 13: 1083-1086.
[16] Cohen MC, Weetman RM, Provisor AJ. Efficacy of magnetic resonance imaging in 139 children with tumors. Arch Surg 1986; 121:
522-529.
[17] Renato F, Paolo V, Girolamo M, Vigano L, Alessandro P, Claudio V, et al. Malignant retroperitoneal teratoma: case report and
literature review. Acta Urol Belg 1996; 64: 49-54.
[8] P.Mathur, M.A.Lopez-Viego, andM.Howell, “Giantprimary retroperitonealteratomainanadult: acasereport,”CaseReports
inMedicine, vol.2010, ArticleID650424, 3pages, 2010
[9] H. Liu, W. Li, W. Yang, and Y. I, “Giant retroperitoneal teratomainanadult,”AmericanJournalofSurgery, vol.193, no. 6, pp.736–
737, 2007.
[10] C. W. Pinson, S. G. ReMine, W. S. Fletcher, and J. W. Braasch, “Long-term results with primary retroperitoneal tumors,”
ArchivesofSurgery, vol.124, no.10, pp.1168–1173, 1989
[19] H. G. Gatcombe, V. Assikis, D. Kooby, and P. A. S. Johnstone, “Primaryretroperitonealteratomas: areviewof theliterature,”
JournalofSurgicalOncology, vol.86, no.2, pp.107–113, 2004.

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Benign Retroperitoneal Teratoma in young adult--A case report and literature review

  • 1. IOSR Journal of Dental and Medical Sciences (IOSR-JDMS) e-ISSN: 2279-0853, p-ISSN: 2279-0861.Volume 14, Issue 11 Ver. IV (Nov. 2015), PP 74-76 www.iosrjournals.org DOI: 10.9790/0853-141147476 www.iosrjournals.org 74 | Page Benign Retroperitoneal Teratoma in young adult--A case report and literature review Dr.Abhishek Kr Singh1 ,Dr.H.I Pandey2 , Dr.Abhay Kumar3 Department of Surgery, Tata Main Hospital, Jamshedpur Abstract: Teratomas are the germ cell tumours which comprises of tissues from all the three germ cell layers. Primary retroperitoneal teratoma is a relatively rare tumour in adults. The primary retroperitoneal teratoma constitutes 6–11% of the retroperitoneal tumours. Sixty percent of the retroperitoneal teratomas occur in children less than 15 years. It occurs more commonly in females than males. The order of frequency for teratoma is ovary, testis, mediastinum and the retroperitoneum at last. Early diagnosis and surgery is the mainstay of treatment. We report a rare case of retroperitoneal teratoma presented to us as left parietal wall abscess. Exploratory laparotomy with tumour resection was performed. Histopathological diagnosis confirmed a benign cystic teratoma. The patient is doing well and is on regular follow up. Keywords: Retroperitoneum, teratoma, laparotomy, mediastinum I. Introduction Teratomas are uncommon neoplasm‟s that contain derivatives of all three germ layers (ectoderm, mesoderm and endoderm). Historically, teratomas were attributed to demons, sexual misconduct and abnormal fertilization. As with teratogenic, the name derives from the Greek word teras, meaning „monster‟. Teratomas belong to a class of tumours known as non-seminomatous germ cell tumour and are typically located in gonadal region. However, extragonadal sites such as the sacrococcygeal region, mediastinum, neck, diancephalon and retroperitoneum have also been reported. Retroperitoneal teratomas often occur in infancy and childhood but are rare in adults. In this article, we describe an unusual case of a retroperitoneal teratoma in a 18-year old male patient who presented as left parietal wall abscess subsequently, underwent successful surgical resection. II. Case Report A 18-year old boy presented with fever on and off for last 1 month and left upper abdominal distension for 15 days. He had no weight loss, bowel or urinary complaints. Mass was aspirated at local hospital and pus like thing came out and later referred here. On examination, a large diffuse mass was palpable in the left flank extending to left of umbilicus. X-ray Abdomen shows dilated bowel loop with calcification. Ultrasound Abdomen shows large cystic lesion of 7x10cm (approx.Vol. 400 cc) with layering of its content is noted in epigastric region. Non Contrast CT abdomen reveal few dilated bowel loops with few bowel entrapment with target sign positive, descending bowel more dilated. Chest X-ray revealed no lung metastases or lymphadenopathy. Laboratory blood investigations shows leucocytosis W.B.C 14,500 and Hb-10.5gm%.Others parameters were within normal limit. Tumour marker AFP, B-HCG and LDH were within normal values. Fig.1 X-ray Abdomen shows calcification with dilated bowel Fig 2.NCCT scan showing mass with the presence of calcification.
  • 2. Benign Retroperitoneal Teratoma in young adult--A case report and literature review DOI: 10.9790/0853-141147476 www.iosrjournals.org 75 | Page In view of above findings he was taken for Emergency exploratory laparotomy.Operative finding revealed large retroperitoneal dermoid tumour (?teratoma mature) with tooth (multiple)with hair and vascular pedicle, tumour and pus like collection of 700 ml.Tumour was anterior to kidney, adherent in retroperitoneal area including mesenteric vessel and adherent to aorta and renal vein and artery in close association with pancreas and portal vein,ureter separate, mesocolon involved but vessels spared. Tumour contains pus and sebum, hair and tooth with capsule. Macroscopically growth measured 8x6x4cm.Microscopically, section show squamous epithelium with intact basement membrane, underneath hair follicle and osteoid material present with areas of haemorrhage and focal chronic inflammatory exudates, few sebaceous glands. Histopathology confirmed the diagnosis of a benign cystic teratoma, benign in nature, with no malignant cells present. Postoperative course was uneventful. After 1 month, in follow up,he is fine with no recurrence or any symptoms. Fig 3.Gross appearance of retroperitoneal mass Fig.4 Microscopic examination of the tumor showing Squamous epithelium, pilosebaceous glands III. Discussion Overall, primary retroperitoneal teratomas constitute about 1-11% [1-3] of all primary retroperitoneal tumours. Approximately half of all teratomas found in children are discovered in first decade of life with 43- 45% of retroperitoneal teratomas diagnosed within the first year of life. Less than 10-20% of retroperitoneal teratomas present in patients after 30 years of age [4]. Teratomas arise from germ cells that fail to mature normally in the gonadal locations. These totipotent cells can differentiate into tissue components representing derivatives of mesoderm, ectoderm and endoderm. The distribution of teratomas listed in order of decreasing frequency is: ovaries, testes, anterior mediastinum, retroperitoneal space, pre sacral and coccygeal areas, pineal and other intracranial sites, neck and abdominal viscera other than gonads. The developmental migratory properties of germ cells would explain teratomas in these extragonadal sites, which generally occur along midline structures [1, 5]. Retroperitoneal teratomas are usually asymptomatic except when compression of the surrounding structures occurs. Patients with compressive symptoms may present with back pain, genitourinary symptoms, gastrointestinal symptoms (abdominal distension, pain, nausea and vomiting), as well as lower extremity or genital enema secondary to lymphatic obstruction [1, 6]. The differential diagnosis of retroperitoneal teratomas include ovarian tumors, renal cysts, adrenal tumours, retroperitoneal fibromas, retroperitoneal sarcomas (usually liposarcomas), hemangiomas, xanthogranulomas, enlarged lymph nodes and perirenal abscesses [7, 8]. Testicular ultrasound is necessary to rule out testicular germ cell tumour in male patients. This is a necessary step since 50% of men with retroperitoneal tumours also have testicular carcinoma in situ, a precursor for testicular germ cell tumours [9]. Calcifications in teratomas seen within the tumour or on the rim of the cyst wall. Even though 74% of benign teratomas contain calcification, they also occur in 12.5% of malignant teratomas [10]. Ultrasound can identify the cystic, solid or complex components of the tumour [11]. The cystic portion may be further differentiated into sebum, non-fat fluid and structures resembling fetal parts. However, ultrasound has its limitations as Davidson et al found that ultrasound poorly identified fat and calcifications, which are suggestive of teratoma. CT gives more specific information on the fat, proteinaceous fluid and calcification components of the teratoma. The presence of fatty portions of the tumour in the horizontal interface with dependent fluid, which probably represents sebum, is virtually pathognomonic of a teratoma [11, 12]. However, a fat fluid level has also
  • 3. Benign Retroperitoneal Teratoma in young adult--A case report and literature review DOI: 10.9790/0853-141147476 www.iosrjournals.org 76 | Page been described in case of a well differentiated liposarcoma of the retroperitoneum [13]. Second, CT appears superior to ultrasound at defining extent in to surrounding organs and for evaluating the cyst wall [11]. MRI is superior to both ultrasound and CT in defining the anatomical relationship of the teratoma with adjacent organs and local tumour spread [14, 15]. MRI can also distinguish fluid, fat, calcium and soft tissue elements, as well as predict resectability and evaluate recurrence [16]. Angiography is beneficial for detecting the presence of hypervascularity, arterial encasement and organ invasion, features often suggesting malignancy [17]. Surgical excision of benign (mature) teratoma is required for a definitive diagnosis (by histopathological examination) [18] and remains the mainstay of treatment [19]. Prognosis is fortunately excellent after complete surgical excision with an overall five-year survival rate of nearly 100% [20]. Teratomas are largely resistant to radio- and chemotherapy. Adjuvant radio- and chemotherapy are used only if malignant features of germ cell tumours are identified on histopathological examination [21]. IV. Conclusion Teratomas can macroscopically be divided into 2 categories: cystic or solid. Cystic teratomas are mostly benign, containing sebaceous materials and mature tissue types. On the other hand, solid teratomas are often malignant and composed of immature embryonic tissues in addition to adipose, cartilaginous, fibrous and bony components. Since it does not respond to chemotherapy and radiotherapy, surgery is the mainstay of treatment. The prognosis is excellent for benign retroperitoneal teratoma if complete resection can be accomplished. Close follow-up is required, as histological mature teratoma may turn malignant. 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