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Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2013, Article ID 610280, 5 pages http://dx.doi.org/10.1155/2013/610280 Case Report Mature (Benign) Cystic Retroperitoneal Teratoma Involving the Left Adrenal Gland in a 22-Year-Old Male: A Case Report and Literature Review 1 2 3 1 4 Adnan Bhatti, Hindi Al-Hindi, Ayman Azzam, Tarek Amin, and Ahmed Abu-Zaid Department of Surgical Oncology, King Faisal Specialist Hospital and Research Center (KFSH&RC), P.O. Box 3354, Riyadh 11211, Saudi Arabia Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center (KFSH&RC), P.O. Box 3354, Riyadh 11211, Saudi Arabia Department of General Surgery, Faculty of Medicine, Alexandria University, Alexandria 21526, Egypt College of Medicine, Alfaisal University, P.O. Box 50927, Riyadh 11533, Saudi Arabia Correspondence should be addressed to Ahmed Abu-Zaid; firstname.lastname@example.org Received 12 April 2013; Accepted 2 May 2013 Academic Editors: E. Itakura and B. I. Razzouk Copyright © 2013 Adnan Bhatti et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Primary retroperitoneal teratomas involving adrenal glands are exceedingly uncommon accounting for only 4% of all primary teratomas. They are more common in childhood and rarely occur in adults. Only a very few case reports have been documented in literature so far. Herein, we report a mature (benign) cystic retroperitoneal teratoma in the region of left adrenal gland in a 22- year-old otherwise healthy male patient who presented with a 1-month history of left flank pain. In addition, a literature review on teratomas is included. 1. Introduction complete surgical excision with an overall vfi e-year survival rate of nearly 100% . Herein, we report a mature (benign) Primary mature teratomas are uncommon nonsemino- cystic retroperitoneal teratoma in the region of left adrenal matous germ cell tumors. eTh y are made up of well- glandina22-year-oldotherwise healthymalepatient who differentiated parenchymal tissues that are derived from more presented with a 1-month history of left flank pain. than one of the three germ cell layers (ectoderm, mesoderm, and endoderm) . They usually occur in midline (paraxial) structures. eTh most common sites are gonads (testes and 2. Case Report ovaries) followed by extragonadal sites such as intracranial, cervical, mediastinal, retroperitoneal, and sacrococcygeal A 22-year-old otherwise healthy male patient presented to regions . Primary retroperitoneal teratomas involving King Faisal Specialist Hospital and Research Centre with a adrenal glands are exceedingly uncommon accounting for 1-month history of left flank pain. Physical examination was only 4% of all primary teratomas [2–4]. They are more remarkable for a palpable, nontender, and limitedly mobile common in childhood and rarely occur in adults . Only mass on the left flank. All laboratory investigations were a very few case reports have been documented in litera- unremarkable. An abdominal contrast-enhanced computed ture so far . eTh majority of cases are asymptomatic, tomography (CT) scan showed a large lesion in the region of present with nonspecific complaints, or identified inciden- left adrenal gland measuring 9.0×9.2×10.8 cm and demon- tally on routine investigations . Surgical excision of mature strating multiple cystic spaces with thin septations as well as (benign) teratoma is required for a definitive diagnosis (by multiple areas of fatty collections and coarse calcifications histopathological examination)  and remains the mainstay (Figure 1(a)). The huge space-occupying lesion displaced of treatment . Prognosis is fortunately excellent aeft r the left kidney inferiorly ( Figure 1(b)). Bearing in mind 2 Case Reports in Oncological Medicine (a) (b) Figure 1: Abdominal contrast-enhanced computed tomography (CT) scan. (a) Cross-sectional (transverse) view: showing a large lesion in the region of left adrenal gland measuring 9.0×9.2×10.8 cm and demonstrating multiple cystic spaces with thin septations as well as multiple areas of fatty collections and coarse calcifications. (b) Coronal (frontal) view: showing displacement of left kidney inferiorly. a potential malignant tumor mass in the retroperitoneum cervical, retroperitoneal, mediastinal, and sacrococcygeal (i.e., liposarcoma), the surgical oncology team advised for a sites [2, 4]. surgical resection. According to the location of tumor, teratomas can be The patient underwent complete surgical resection of the classiefi d into gonadal and extragonadal teratomas. Gonadal left adrenal mass. Grossly, the left adrenal mass weighted teratomas are more common, mostly primary neoplasms, 484 g and measured 9.0 × 9.0 × 10 cm. eTh mass had mainly in adults, and usually take place in gonads (testes well-circumscribed smooth borders and rubbery consis- and ovaries) . Conversely, extragonadal teratomas are less tency. Cut-section of the mass revealed multilocular cystic common, mostly secondary neoplasms, mainly in infants and spaces, whitish-gray walls, scattered yellowish adipose tissue young children , and usually take place in sacrococcygeal, collections, mucus secretions, and areas of calcicfi ations mediastinal, retroperitoneal, and pineal gland sites (descend- (Figure 2(a)). Microscopically, the walls were largely lined by ing order of frequency) [11–13]. respiratory columnar and squamous epithelium with various Furthermore, according to the content of tumor, ter- proportions of mature well-differentiated parenchymal tis- atomas can be classified into solid, cystic, or mixed ter- sues derived from the various three germ cell layers (Figures atomas. Solid teratomas lack organization and contain only 2(b) and 2(c)). No evidence of malignancy was identified. A parenchymal tissues. Cystic teratomas contain only sacs of diagnosis of mature (benign) cystic retroperitoneal teratoma u fl id, semifluid, or fat, whereas mixed teratomas contain both involving the left adrenal gland was made. solid and cystic components . Since there was no evidence of immature or malignant Besides, according to the epithelial lining and dermal components, no radio- or chemotherapy was oer ff ed. eTh contents of tumor, teratomas can be classified into epider- patient was discharged uneventfully in a stable condition. A moid, dermoid, and teratoid teratomas (cysts). Epidermoid postoperative 6-month followup failed to show any evidence teratomas are lined by stratiefi d squamous epithelium and of tumor recurrence. lack dermal contents. Dermoid teratomas are mostly lined by stratiefi d squamous epithelium and contain various dermal contents such as hair, sweat, and sebaceous glands. Teratoid 3. Discussion teratomas are mostly lined by respiratory columnar epithe- lium and contain sebum . Germ cell tumors (GCTs) can be broadly classified into In addition, according to the degree of tumor matura- two main categories: seminomatous and nonseminomatous tion, teratomas can be classiefi d into mature and immature GCTs. Teratomas belong to nonseminomatous GCTs and rep- teratomas. Mature teratomas are generally benign, asymp- resent themostcommonformofall GCTs . Teratomas are tomatic and more common, among females. They are highly encapsulated neoplasms composed of multiple parenchymal variable on histology and can be solid, cystic, or mixed. tissues (of varying degrees of differentiation) that are derived They contain different types of parenchymal tissues that are from more than one germ cell layer (ectoderm, mesoderm, well differentiated. Mature cystic teratomas (AKA dermoid and endoderm) . Generally, teratomas arise from uncontrolled prolifera- cysts) may have partially to completely well-developed organ tion of pluripotent cells: germ cells and embryonal cells. eTh systems. On the contrary, immature teratomas are histolog- type of pluripotent cell greatly influences the presentation ically solid teratomas and contain immature (undifferenti- ated/undeveloped) parenchymal tissues and can be possibly time andinvolvedlocationofteratoma. Teratomasofgerm cell sources can be congenital or acquired and are usually benign, possibly malignant, or frankly malignant. They are found in gonads (testes and ovaries). In contrast, teratomas more common among males [8, 14]. Some mature (benign) and immature (possibly benign or of embryonic cell sources are always congenital and are usually found in extragonadal locations, such as intracranial, possibly malignant) teratomas have an increased tendency to Case Reports in Oncological Medicine 3 0 123456789 10 (cm) (a) L C (b) (c) Figure 2: Mature (benign) cystic retroperitoneal teratoma in the region of left adrenal gland. (a) Grossly, cut-section of the resected mass revealed multilocular cystic spaces, whitish-gray walls, scattered yellowish adipose tissues, mucus secretions, and areas of calcifications. (b) Hematoxylin & Eosin (H&E stain),×20 magnification: a scanning magnification view of the lesion depicting its relationship with the adrenal cortex (open arrows). eTh lumen [L] is lined by respiratory columnar epithelium. (c) Hematoxylin & Eosin (H&E stain), ×40 magnification: a low magnification image of another cystic space lined by mucous-secret ing epithelium (solid arrows). The wall is formed by cartilage tissues [C], fat (adipose) tissues [F], and salivary gland tissues (open arrows). become frankly malignant teratomas, and frankly malignant syndrome (GTS). GTS refers to a rapidly growing pure teratomas have an increased propensity to metastasize. This mature (benign) teratoma that appears during or following group of exceptionally rare teratomas is known as teratomas chemotherapeutic eradication of malignant components of with malignant transformation . The stratified squamous a nonseminomatous germ cell tumor, and it has normal epithelial components of these teratomas are the ones at serum tumor marker levels of alpha-fetoprotein and human an increased risk of undergoing malignant transformations. chorionic gonadotropin . In addition, teratomas with malignant transformation may eTh vast majority of retroperitoneal teratomas are sec- produce components of somatic (non-germ cell) neoplasms ondary neoplasms and mostly occur in males . Primary such as carcinoma, sarcoma, and leukemia [15, 16]. retroperitoneal teratomas are extremely unusual neoplasms Occasionally, a teratoma may contain various compo- accounting for approximately 1–11% of all primary retroperi- nents of other germ cell tumor, and hence it is not a pure ter- toneal neoplasms and typically occur in neonates, infants, atoma per se, but rather it is a mixed germ cell tumor and has and children age groups . In adults, these neoplasms malignant nature. In infants and young children, these com- commonly present in the third or fourth decades of lives . ponents are frequently endodermal sinus tumor and chorio- Primary retroperitoneal teratomas involving adrenal carcinoma. A pure teratoma can be benign, however, highly glands are exceedingly uncommon accounting for only 4% aggressive in its clinical course as in a growing teratoma of all primary teratomas [2–4]and canbemistakenfor other 4 Case Reports in Oncological Medicine histologically related lipomatous adrenal neoplasms . They teratomas), and its occurrence in an adult is exceptionally aremorecommoninchildhoodandrarelyoccurinadults. uncommon. However, it should be regarded in the dif- Only a few case reports have been documented in ferential diagnosis in any patient presenting with a flank literature so far . eTh y are more frequently encountered at pain. Histopathological examination of the resected tumor the left side [ 3, 4]. eTh majority of cases are asymptomatic, warrants a definitive diagnosis. Surgical excision of mature present with nonspecific complaints, or identified inciden- (benign) teratoma remains the mainstay of treatment with an tally on routine investigations . excellent vfi e-year survival rate of nearly 100%. Teratomas can be diagnosed based on high index of clinical suspicion, routine laboratory, and radiographic inves- Acknowledgment tigations . With respect to high index of clinical sus- picion, retroperitoneal teratomas involving adrenal glands eTh authors sincerely acknowledge the editorial assistance may present congenitally, or later in life when they grow of Ms. Ranim Chamseddin, College of Medicine, Alfaisal to massive sizes . Clinical presentations are variable and University, Riyadh, Saudi Arabia. include nonspecific, abdominal/flank/back pain, obstructive gastrointestinal and genitourinary symptoms, as well as lower References limb/genital swelling due to lymphatic obstruction . They can rarely present with complications such as secondary  D. J. B. 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