APMIS
© 2014 APMIS. Published by John Wiley & Sons Ltd. DOI 10.1111/apm.12216
Paediatric urachal benign teratoma: a case report and review of the literature PERNILLE SKJOLD KINGO,1 SØREN HØYER,2 EDVARD MARINOVSKIJ3 and YAZAN FAIZ RAWASHDEH1 Departments of Urology; 2Pathology; 3Diagnostic Imaging, Aarhus University Hospital, Skejby, Denmark
1
Kingo PS, Høyer S, Marinovskij E, Rawashdeh YF. Paediatric urachal benign teratoma: a case report and review of the literature. APMIS 2014. Urachal anomalies are most often seen in children, seldom in adults, but are in general considered rare. The estimated incidence is one in 5000–7000 live births and appears twice as common in males. Despite their rarity, they need to be considered by clinicians, as diseases in the urachus can mimic many abdominal and pelvic conditions and constitute an important differential diagnosis to these. Diagnosis can be made by clinical examination and imaging modalities (computed tomography, ultrasonography, magnetic resonance imaging, voiding cystourethrogram), but some are discovered incidentally. Management of symptomatic urachal anomalies is surgery. Histological examination of the specimen should always be performed to rule out malignancy. We report on the first adolescent described in the literature diagnosed with a urachal sinus harboring a benign teratoma. A combination of the two pathologies is by inference an extremely rare condition, which we here report on and we review the relevant literature on this topic. Key words: Pediatric; urachal remnants; pathology; urachal teratoma; treatment. Pernille Skjold Kingo, Aarhus University Hospital, Department of Urology, Brendstrupgaardsvej 100, DK-8200 Aarhus N, Denmark. e-mail: [email protected]
Urachal anomalies are most often seen in children, seldom in adults (1), but in general, urachal remnants are considered rare (2). The estimated incidence is one in 5000–7000 live births (3). Despite their rarity, they need to be considered by clinicians and especially urologists, as diseases in the urachus can mimic many abdominal and pelvic conditions and constitute an important differential diagnosis to these. The diagnosis can be made by imaging modalities such as ultrasonography (US), computed tomography (CT), voiding cystourethrogram or fistulography, and Magnetic resonance imaging (MRI) (1–5). In the following, we present the first adolescent described in the literature diagnosed with a benign teratoma found in a urachal remnant and we review on the relevant literature on this topic. CASE REPORT A 14-year-old Caucasian girl known to have neurofibromatosis was investigated for recurrent diffuse Received 8 August 2013. Accepted 21 October 2013
periumbilical pain and lower urinary tract infections (UTI), which had been ongoing for a period of 3–4 years prior to presentation. Worsening of pain was experienced in conjunction with menstrual periods and food intake. Also a history of occasional episodes of self-limiting macroscopic hematuria was observed. In addition, there was a history of intermittent umbilical discharge. Clinical examination was unremarkable, revealing no abdominal tenderness and the umbilicus appeared normal. Based on the history, a urachal anomaly was suspected and an MRI-scan was performed. This revealed a persistent fluid-filled urachal sinus with obliteration of the rest of the urachus (Fig. 1). The urachal sinus was surgically removed by open extraperitoneal excision under general anesthesia. The histopathology of the specimen revealed a urachus, measuring 9 cm in length, with a diameter ranging from 5 to 7 mm. Macroscopically, a teratoma was not seen, but microscopy showed a duct, which was lined by urothelium close to the bladder, and surrounded by smooth muscle cells. Further caudally, a transition to a non-keratinized squamous epithelium was seen. At the fascial end 1
KINGO et al.
Fig. 1. Magnetic resonance imaging (MRI), Sagittal T2weighted image show persistent fluid-filled urachal sinus (asterisk). The rest of the urachus (arrow) is obliterated.
toward the bladder, the squamous epithelium became keratinized, and in the underlying stroma, mature ducts and complete mature sweat glands were found, imitating normal skin (Fig. 2). There were no immature elements, and the teratoma was of a pure ectodermal and mesodermal type. On follow-up 6 months later, the patient had no longer abdominal pain, nor recurrent lower UTI. Only complaint was of cosmetic concern with hypertrophic scar formation, which was adequately managed by application of mild Steroid cream.
DISCUSSION The Urachus is an allantoic remnant located between the umbilicus and the dome of the bladder.
It lies in the space of Retzius behind the transversalis fascia and anterior to the peritoneum. During the fourth and fifth gestational month, the fetal bladder descends from the umbilicus to the pelvis causing the allantois to stretch and progressively narrow to an epithelialized fibromuscular tubular structure. Usually, the lumen obliterates by fibrous proliferation and remains as a fibrous band – the urachus. Histologically, the urachus is composed of all three bladder layers: An Innermost layer lined with transitional epithelium (70%) or columnar epithelium (30%) surrounded by connective tissue and an outermost muscular layer in continuum with the detrusor muscle (1, 6). Incomplete involution of the urachus results in urachal anomalies, which can be divided into five main types of persistent urachal remnants according to the patency of the duct (1, 5, 7) (Table 1). Urachal cyst, sinus, and diverticulum may be closed at birth, but can reopen at pathological conditions as for instance seen in untreated infected urachal cysts that spontaneously rupture and drain via the umbilicus and/or into the bladder. Therefore, they are sometimes referred to as acquired diseases, most commonly presenting in adulthood (1, 2, 8). In general, urachal anomalies are rare, with an estimated incidence of one in 5000–7000 live births being twice as common in males (3). Clinical presentation and symptomatology varies depending on the type of urachal anomaly; however, some are discovered incidentally on imaging. Most common signs and symptoms reported in the literature are midline palpable mass, symptoms of UTI, expression of urine at the umbilicus, umbilical (purulent) discharge, periumbilical erythema, and abdominal pain mimicking other pathological abdominal conditions (1–5, 7, 8). Management of symptomatic urachal anomalies is surgery by open extraperitoneal dissection of the urachal tract, with excision of the tract from the base of the umbilicus to the dome bladder including a small bladder cuff (5, 7, 8). Laparoscopic removal can also be performed (5). All removed specimen
Fig. 2. Urachus lined by keratinized squamous epithelium with underlying dermal looking connective tissue, including numerous eccrine sweet-like glands and collecting ducts.
2
© 2014 APMIS. Published by John Wiley & Sons Ltd
URACHAL BENIGN TERATOMA
Table 1. Urachal Anomalies Type Anatomical description Patent urachus Persistent duct between the umbilicus and the dome of the bladder A variant is vesicoumbilical fistula, resulting from failure of the bladder to descent [2]
Illustration
Occurence [2,4] 10–48%
Urachal cyst
A cyst along the urachal duct, which is mainly located in the middle of the duct, which has obliterated cranially and caudally
31–43%
Urachal sinus
In an umbilicourachal sinus, only the cranial infra umbilical part of the duct is open, whereas the rest is closed
18–43%
Alternating sinus
A cystlike structure that can drain either into the umbilicus or the bladder [2]
Rare
Urachal diverticulum
The caudal part of the duct is open, whereas the rest of the duct is closed creating an extension of the bladders dome
3–4%
*umbilicus †bladder ‡urethra
should be examined histologically to rule out malignancy, bearing in mind that urachus adenocarcinoma can arise from preexisting urachal remnants and involving the bladder by infiltration. It is though noteworthy that malignancy in urachal remnants is rarely diagnosed in children, but in the adult population, adenocarcinomas of urachal remnants accounts for 1% of all bladder carcinomas. It has been discussed in the literature whether or not urachal remnants should be removed when found incidentally knowing that some studies have shown that advancing age is associated with increased risk of developing urachal adenocarcinoma. Whether observation of pediatric remnants is safe is difficult to say as congenital urachal remnants are rare, which makes prospective studies difficult. However, given the poor prognosis associated with urachal adenocarcinomas (5-year survival little less than 50%), it is suggested that all recognized pediatric remnants should be removed as the ability to pre© 2014 APMIS. Published by John Wiley & Sons Ltd
dict malignant transformation is poor and the morbidity associated with surgical excision is low (9).Other pathologic conditions found in urachus anomalies presented in case reports only are rhabdomyosarcoma, angiomatous leiomyoma, neuroendocrine carcinoma, and fibrous histiocytoma. As with urachal anomalies, teratomas are also uncommon. Teratomas are benign germ cell neoplasms composed of tissue derived from the endoderm, mesoderm, and ectoderm of the embryo usually foreign to the anatomic site of origin. Teratomas can originate anywhere along the midline, but are most commonly seen in the sacrococcygeal, gonadal, mediastinal, retroperitoneal, cervicofacial, and intracranial locations. Extragonadal teratomas are most often seen in neonates and in children, whereas gonadal tumors are more common in adolescents. Nearly 80% of all teratomas occur in females and no racial predilection has been proven (10). Teratomas are classified as mature or immature
3
KINGO et al.
Table 2. Overview of case reports on urachal remnants harboring teratomas Reference Gender Location Age Histology Size (cm) Symptoms
Therapy
(12)
Female
Urachal sinus
8
Benign
Small apple
(13)
Female
Urachal cyst
53
Benign
35 9 20
(14)
Female
Urachal cyst
36
Benign
99695
(15)
Female
20
Benign
8 9 4.5
(16)
Female
Urachal cyst Urachal cyst
Umbilical infection and purulent discharge Umbilical infection, Abdominal pain, extension of the abdomen Rec UTI, suprapubic pain Rec UTI
27
Benign
–
Current case
Female
14
Benign
9 9 0.7
Pelvic pain, abdominal umbilical mass Rec UTI, periumbilical pain, umbilical discharge
Urachal sinus
(potentially malignant) with most pediatric tumors being mature. Symptomatology depends on the anatomical location and size. The diagnosis can be made from clinical examinations combined with imaging techniques (CT, MRI and US). (10, 11). Knowing that urachal anomalies and teratomas each constitute a rarity, a combination of the two pathologies is thus an extremely rare condition. So much so, that a review of the literature only revealed five reported cases prior to the current one. In 1963, Revel F et al. reported in French on the first case of an 8-year-old girl with a benign teratoma found in a urachal remnant containing a Bilharzian granuloma (12). The second case was reported by Cionfoli et al. in 1991 in Italian (13). Both tumors were successfully removed and the patients rendered asymptomatic. Subsequently, three more cases of urachal teratomas have been reported in the literature, two in English (1998 and 2006) (14, 15) and another one in the French literature (2006) (16) (Table 2). Of the six total reported cases, all patients were female. Four of the six reported cases were adult patients, making the current case the second to be reported in the pediatric age group and the first adolescent. Teratoma size ranged from 8 to 35 cm with the dominant symptomatology being recurring lower UTI and abdominal pain mainly periumbilical. Umbilical discharge was only seen in the pediatric 4
Other
Surgery
Followup None
Surgery
None
–
Surgery
None
None
Surgery
None
None
Surgery
None
Encephalitis with sequelae of epilepsy
Surgery
6 months
Neurofibromatosis
Schistosomiasis
cases. Diagnosis of the urachal anomaly was made by US supplemented by a CT-scan in three of the cases (13–15) and in two cases, including the current one, also an MRI (14). Radiographic imaging revealed in the majority of cases a cystic mass in close correlation with the urachus. Only in one case was the diagnosis of a urachal teratoma made by CT-scan, which revealed high-density structures in the mass resembling teeth (15). In the remaining cases, the diagnosis of a urachus harboring a teratoma was not revealed until histological examination. The urachal remnant and teratoma was in all six cases surgically excised in toto. Histopathology showed that all specimens were urachal remnants harboring mature teratomas with no evidence of malignancy. The malignant potential of teratomas in urachal remnants remains unknown due to the small number of cases and limited follow-up. It is, however, noteworthy that patients treated for mature benign teratomas per se have an excellent prognosis. CONCLUSION Urachal anomalies are rare in children, but the diagnosis remains relevant to clinicians and especially urologists in the differential diagnosis of abdominal pain and recurrent lower urinary tract infection. The diagnosis is easily made by clinical © 2014 APMIS. Published by John Wiley & Sons Ltd
URACHAL BENIGN TERATOMA
examination supplemented by imaging techniques. Treatment of choice is surgical excision. Histological examination of the specimen should always be performed to rule out malignancy or to identify the rarer and, at times, more exotic variants as in the current case. CONFLICT OF INTEREST No conflict of interest.
REFERENCES 1. Nimmorat A, Na-ChiangMai W, Muttarak M. Urachal abnormalities: clinical and imaging features. Singapore Med J 2008;49:930–5. 2. Widni EE, H€ ollwarth ME, Haxhija EQ. The impact of preoperative ultrasound on correct diagnosis of urachal remnants in children. J Pediatr Surg 2010; 45: 1433–7. 3. Austin PF. Umbilical and urachal anomalies. In: Wilcox DT, Godbole PP, Koyle MA, editors. Pediatric Urology: Surgical Complications and Management. Oxford: Blackwell Publishing Ltd., 2008: 92–7. 4. Yiee JH, Garcia N, Baker LA, Barber R, Snodgrass WT, Wilcox DT. A diagnostic algorithm for urachal anomalies. J Pediatr Urol 2007;3:500–4. 5. McCollum MO, MacNeily AE, Blair GK. Surgical implications of urachal remnants: presentation and management. J Pediatr Surg 2003;38:798–803.
© 2014 APMIS. Published by John Wiley & Sons Ltd
6. Begg RC. The Urachus: its anatomy, histology and development. J Anat 1930;64:170–83. 7. Mesrobian HG, Zacharias A, Balcom AH, Cohen RD. Ten years of experience with isolated urachal anomalies in children. J Urol 1997;158:1316–8. 8. Schlaishunt S, Rubin J. A case of Urachal Remnant presenting as acute abdominal pain. J Emer Med 1999;17:243–6. 9. Ashley RA, Inman BA, Routh JC, Rohlinger AL, Husmann DA, Kramer SA. Urachal anomalies: a longitudinal study of urachal remnants in children and adults. J Urol 2007;178:1615–8. 10. Barksdale EM Jr, Obokhare I. Teratomas in infants and children. Cur Opin Pediatr 2009;21:344–9. 11. Gatcombe HG, Assikis V, Kooby D, Johnstone PAS. Primary retroperitoneal teratomas: a review of the literature. J Surg Oncol 2004;86:107–13. 12. Revel F, Beziade R, Peloux Y. Apropos of a teratoma of the umbilicus invaded by a bilharzian granuloma. Medicine Tropicale 1963;23:412–6 (article in French) 13. Cionfoli U, Malaspina C, Ciceri P, Capriata G, Battaini A, Macchi R, et al. A case of teratoma of the urachus. Minerva Chir 1991;46:471–7 (article in Italian) 14. Defibiani N, Iselin CE, Khan HG, Pache JC, Rohner S. Benign teratoma of the urachus. Brit J Urol 1998;81:760–1. 15. Lang EK, Castle E, Macchia R, Colon I. Computerized tomography diagnosis of urachal cyst harboring teratoma. J Urol 2006;176:1623. 16. Agbreta N, Boutens A, Debodinance P. Kystedermo€ıde de l’ouraque. A propos d’un cas et revue de la literature. J Gynecol Obstet Biol Reprod 2006;35:75–8 (article in French)
5
© 2014 APMIS. Published by John Wiley & Sons Ltd. DOI 10.1111/apm.12216
Paediatric urachal benign teratoma: a case report and review of the literature PERNILLE SKJOLD KINGO,1 SØREN HØYER,2 EDVARD MARINOVSKIJ3 and YAZAN FAIZ RAWASHDEH1 Departments of Urology; 2Pathology; 3Diagnostic Imaging, Aarhus University Hospital, Skejby, Denmark
1
Kingo PS, Høyer S, Marinovskij E, Rawashdeh YF. Paediatric urachal benign teratoma: a case report and review of the literature. APMIS 2014. Urachal anomalies are most often seen in children, seldom in adults, but are in general considered rare. The estimated incidence is one in 5000–7000 live births and appears twice as common in males. Despite their rarity, they need to be considered by clinicians, as diseases in the urachus can mimic many abdominal and pelvic conditions and constitute an important differential diagnosis to these. Diagnosis can be made by clinical examination and imaging modalities (computed tomography, ultrasonography, magnetic resonance imaging, voiding cystourethrogram), but some are discovered incidentally. Management of symptomatic urachal anomalies is surgery. Histological examination of the specimen should always be performed to rule out malignancy. We report on the first adolescent described in the literature diagnosed with a urachal sinus harboring a benign teratoma. A combination of the two pathologies is by inference an extremely rare condition, which we here report on and we review the relevant literature on this topic. Key words: Pediatric; urachal remnants; pathology; urachal teratoma; treatment. Pernille Skjold Kingo, Aarhus University Hospital, Department of Urology, Brendstrupgaardsvej 100, DK-8200 Aarhus N, Denmark. e-mail: [email protected]
Urachal anomalies are most often seen in children, seldom in adults (1), but in general, urachal remnants are considered rare (2). The estimated incidence is one in 5000–7000 live births (3). Despite their rarity, they need to be considered by clinicians and especially urologists, as diseases in the urachus can mimic many abdominal and pelvic conditions and constitute an important differential diagnosis to these. The diagnosis can be made by imaging modalities such as ultrasonography (US), computed tomography (CT), voiding cystourethrogram or fistulography, and Magnetic resonance imaging (MRI) (1–5). In the following, we present the first adolescent described in the literature diagnosed with a benign teratoma found in a urachal remnant and we review on the relevant literature on this topic. CASE REPORT A 14-year-old Caucasian girl known to have neurofibromatosis was investigated for recurrent diffuse Received 8 August 2013. Accepted 21 October 2013
periumbilical pain and lower urinary tract infections (UTI), which had been ongoing for a period of 3–4 years prior to presentation. Worsening of pain was experienced in conjunction with menstrual periods and food intake. Also a history of occasional episodes of self-limiting macroscopic hematuria was observed. In addition, there was a history of intermittent umbilical discharge. Clinical examination was unremarkable, revealing no abdominal tenderness and the umbilicus appeared normal. Based on the history, a urachal anomaly was suspected and an MRI-scan was performed. This revealed a persistent fluid-filled urachal sinus with obliteration of the rest of the urachus (Fig. 1). The urachal sinus was surgically removed by open extraperitoneal excision under general anesthesia. The histopathology of the specimen revealed a urachus, measuring 9 cm in length, with a diameter ranging from 5 to 7 mm. Macroscopically, a teratoma was not seen, but microscopy showed a duct, which was lined by urothelium close to the bladder, and surrounded by smooth muscle cells. Further caudally, a transition to a non-keratinized squamous epithelium was seen. At the fascial end 1
KINGO et al.
Fig. 1. Magnetic resonance imaging (MRI), Sagittal T2weighted image show persistent fluid-filled urachal sinus (asterisk). The rest of the urachus (arrow) is obliterated.
toward the bladder, the squamous epithelium became keratinized, and in the underlying stroma, mature ducts and complete mature sweat glands were found, imitating normal skin (Fig. 2). There were no immature elements, and the teratoma was of a pure ectodermal and mesodermal type. On follow-up 6 months later, the patient had no longer abdominal pain, nor recurrent lower UTI. Only complaint was of cosmetic concern with hypertrophic scar formation, which was adequately managed by application of mild Steroid cream.
DISCUSSION The Urachus is an allantoic remnant located between the umbilicus and the dome of the bladder.
It lies in the space of Retzius behind the transversalis fascia and anterior to the peritoneum. During the fourth and fifth gestational month, the fetal bladder descends from the umbilicus to the pelvis causing the allantois to stretch and progressively narrow to an epithelialized fibromuscular tubular structure. Usually, the lumen obliterates by fibrous proliferation and remains as a fibrous band – the urachus. Histologically, the urachus is composed of all three bladder layers: An Innermost layer lined with transitional epithelium (70%) or columnar epithelium (30%) surrounded by connective tissue and an outermost muscular layer in continuum with the detrusor muscle (1, 6). Incomplete involution of the urachus results in urachal anomalies, which can be divided into five main types of persistent urachal remnants according to the patency of the duct (1, 5, 7) (Table 1). Urachal cyst, sinus, and diverticulum may be closed at birth, but can reopen at pathological conditions as for instance seen in untreated infected urachal cysts that spontaneously rupture and drain via the umbilicus and/or into the bladder. Therefore, they are sometimes referred to as acquired diseases, most commonly presenting in adulthood (1, 2, 8). In general, urachal anomalies are rare, with an estimated incidence of one in 5000–7000 live births being twice as common in males (3). Clinical presentation and symptomatology varies depending on the type of urachal anomaly; however, some are discovered incidentally on imaging. Most common signs and symptoms reported in the literature are midline palpable mass, symptoms of UTI, expression of urine at the umbilicus, umbilical (purulent) discharge, periumbilical erythema, and abdominal pain mimicking other pathological abdominal conditions (1–5, 7, 8). Management of symptomatic urachal anomalies is surgery by open extraperitoneal dissection of the urachal tract, with excision of the tract from the base of the umbilicus to the dome bladder including a small bladder cuff (5, 7, 8). Laparoscopic removal can also be performed (5). All removed specimen
Fig. 2. Urachus lined by keratinized squamous epithelium with underlying dermal looking connective tissue, including numerous eccrine sweet-like glands and collecting ducts.
2
© 2014 APMIS. Published by John Wiley & Sons Ltd
URACHAL BENIGN TERATOMA
Table 1. Urachal Anomalies Type Anatomical description Patent urachus Persistent duct between the umbilicus and the dome of the bladder A variant is vesicoumbilical fistula, resulting from failure of the bladder to descent [2]
Illustration
Occurence [2,4] 10–48%
Urachal cyst
A cyst along the urachal duct, which is mainly located in the middle of the duct, which has obliterated cranially and caudally
31–43%
Urachal sinus
In an umbilicourachal sinus, only the cranial infra umbilical part of the duct is open, whereas the rest is closed
18–43%
Alternating sinus
A cystlike structure that can drain either into the umbilicus or the bladder [2]
Rare
Urachal diverticulum
The caudal part of the duct is open, whereas the rest of the duct is closed creating an extension of the bladders dome
3–4%
*umbilicus †bladder ‡urethra
should be examined histologically to rule out malignancy, bearing in mind that urachus adenocarcinoma can arise from preexisting urachal remnants and involving the bladder by infiltration. It is though noteworthy that malignancy in urachal remnants is rarely diagnosed in children, but in the adult population, adenocarcinomas of urachal remnants accounts for 1% of all bladder carcinomas. It has been discussed in the literature whether or not urachal remnants should be removed when found incidentally knowing that some studies have shown that advancing age is associated with increased risk of developing urachal adenocarcinoma. Whether observation of pediatric remnants is safe is difficult to say as congenital urachal remnants are rare, which makes prospective studies difficult. However, given the poor prognosis associated with urachal adenocarcinomas (5-year survival little less than 50%), it is suggested that all recognized pediatric remnants should be removed as the ability to pre© 2014 APMIS. Published by John Wiley & Sons Ltd
dict malignant transformation is poor and the morbidity associated with surgical excision is low (9).Other pathologic conditions found in urachus anomalies presented in case reports only are rhabdomyosarcoma, angiomatous leiomyoma, neuroendocrine carcinoma, and fibrous histiocytoma. As with urachal anomalies, teratomas are also uncommon. Teratomas are benign germ cell neoplasms composed of tissue derived from the endoderm, mesoderm, and ectoderm of the embryo usually foreign to the anatomic site of origin. Teratomas can originate anywhere along the midline, but are most commonly seen in the sacrococcygeal, gonadal, mediastinal, retroperitoneal, cervicofacial, and intracranial locations. Extragonadal teratomas are most often seen in neonates and in children, whereas gonadal tumors are more common in adolescents. Nearly 80% of all teratomas occur in females and no racial predilection has been proven (10). Teratomas are classified as mature or immature
3
KINGO et al.
Table 2. Overview of case reports on urachal remnants harboring teratomas Reference Gender Location Age Histology Size (cm) Symptoms
Therapy
(12)
Female
Urachal sinus
8
Benign
Small apple
(13)
Female
Urachal cyst
53
Benign
35 9 20
(14)
Female
Urachal cyst
36
Benign
99695
(15)
Female
20
Benign
8 9 4.5
(16)
Female
Urachal cyst Urachal cyst
Umbilical infection and purulent discharge Umbilical infection, Abdominal pain, extension of the abdomen Rec UTI, suprapubic pain Rec UTI
27
Benign
–
Current case
Female
14
Benign
9 9 0.7
Pelvic pain, abdominal umbilical mass Rec UTI, periumbilical pain, umbilical discharge
Urachal sinus
(potentially malignant) with most pediatric tumors being mature. Symptomatology depends on the anatomical location and size. The diagnosis can be made from clinical examinations combined with imaging techniques (CT, MRI and US). (10, 11). Knowing that urachal anomalies and teratomas each constitute a rarity, a combination of the two pathologies is thus an extremely rare condition. So much so, that a review of the literature only revealed five reported cases prior to the current one. In 1963, Revel F et al. reported in French on the first case of an 8-year-old girl with a benign teratoma found in a urachal remnant containing a Bilharzian granuloma (12). The second case was reported by Cionfoli et al. in 1991 in Italian (13). Both tumors were successfully removed and the patients rendered asymptomatic. Subsequently, three more cases of urachal teratomas have been reported in the literature, two in English (1998 and 2006) (14, 15) and another one in the French literature (2006) (16) (Table 2). Of the six total reported cases, all patients were female. Four of the six reported cases were adult patients, making the current case the second to be reported in the pediatric age group and the first adolescent. Teratoma size ranged from 8 to 35 cm with the dominant symptomatology being recurring lower UTI and abdominal pain mainly periumbilical. Umbilical discharge was only seen in the pediatric 4
Other
Surgery
Followup None
Surgery
None
–
Surgery
None
None
Surgery
None
None
Surgery
None
Encephalitis with sequelae of epilepsy
Surgery
6 months
Neurofibromatosis
Schistosomiasis
cases. Diagnosis of the urachal anomaly was made by US supplemented by a CT-scan in three of the cases (13–15) and in two cases, including the current one, also an MRI (14). Radiographic imaging revealed in the majority of cases a cystic mass in close correlation with the urachus. Only in one case was the diagnosis of a urachal teratoma made by CT-scan, which revealed high-density structures in the mass resembling teeth (15). In the remaining cases, the diagnosis of a urachus harboring a teratoma was not revealed until histological examination. The urachal remnant and teratoma was in all six cases surgically excised in toto. Histopathology showed that all specimens were urachal remnants harboring mature teratomas with no evidence of malignancy. The malignant potential of teratomas in urachal remnants remains unknown due to the small number of cases and limited follow-up. It is, however, noteworthy that patients treated for mature benign teratomas per se have an excellent prognosis. CONCLUSION Urachal anomalies are rare in children, but the diagnosis remains relevant to clinicians and especially urologists in the differential diagnosis of abdominal pain and recurrent lower urinary tract infection. The diagnosis is easily made by clinical © 2014 APMIS. Published by John Wiley & Sons Ltd
URACHAL BENIGN TERATOMA
examination supplemented by imaging techniques. Treatment of choice is surgical excision. Histological examination of the specimen should always be performed to rule out malignancy or to identify the rarer and, at times, more exotic variants as in the current case. CONFLICT OF INTEREST No conflict of interest.
REFERENCES 1. Nimmorat A, Na-ChiangMai W, Muttarak M. Urachal abnormalities: clinical and imaging features. Singapore Med J 2008;49:930–5. 2. Widni EE, H€ ollwarth ME, Haxhija EQ. The impact of preoperative ultrasound on correct diagnosis of urachal remnants in children. J Pediatr Surg 2010; 45: 1433–7. 3. Austin PF. Umbilical and urachal anomalies. In: Wilcox DT, Godbole PP, Koyle MA, editors. Pediatric Urology: Surgical Complications and Management. Oxford: Blackwell Publishing Ltd., 2008: 92–7. 4. Yiee JH, Garcia N, Baker LA, Barber R, Snodgrass WT, Wilcox DT. A diagnostic algorithm for urachal anomalies. J Pediatr Urol 2007;3:500–4. 5. McCollum MO, MacNeily AE, Blair GK. Surgical implications of urachal remnants: presentation and management. J Pediatr Surg 2003;38:798–803.
© 2014 APMIS. Published by John Wiley & Sons Ltd
6. Begg RC. The Urachus: its anatomy, histology and development. J Anat 1930;64:170–83. 7. Mesrobian HG, Zacharias A, Balcom AH, Cohen RD. Ten years of experience with isolated urachal anomalies in children. J Urol 1997;158:1316–8. 8. Schlaishunt S, Rubin J. A case of Urachal Remnant presenting as acute abdominal pain. J Emer Med 1999;17:243–6. 9. Ashley RA, Inman BA, Routh JC, Rohlinger AL, Husmann DA, Kramer SA. Urachal anomalies: a longitudinal study of urachal remnants in children and adults. J Urol 2007;178:1615–8. 10. Barksdale EM Jr, Obokhare I. Teratomas in infants and children. Cur Opin Pediatr 2009;21:344–9. 11. Gatcombe HG, Assikis V, Kooby D, Johnstone PAS. Primary retroperitoneal teratomas: a review of the literature. J Surg Oncol 2004;86:107–13. 12. Revel F, Beziade R, Peloux Y. Apropos of a teratoma of the umbilicus invaded by a bilharzian granuloma. Medicine Tropicale 1963;23:412–6 (article in French) 13. Cionfoli U, Malaspina C, Ciceri P, Capriata G, Battaini A, Macchi R, et al. A case of teratoma of the urachus. Minerva Chir 1991;46:471–7 (article in Italian) 14. Defibiani N, Iselin CE, Khan HG, Pache JC, Rohner S. Benign teratoma of the urachus. Brit J Urol 1998;81:760–1. 15. Lang EK, Castle E, Macchia R, Colon I. Computerized tomography diagnosis of urachal cyst harboring teratoma. J Urol 2006;176:1623. 16. Agbreta N, Boutens A, Debodinance P. Kystedermo€ıde de l’ouraque. A propos d’un cas et revue de la literature. J Gynecol Obstet Biol Reprod 2006;35:75–8 (article in French)
5
