|Year : 2015 | Volume
| Issue : 3 | Page : 647
Unusual presentation of immature teratoma of the neck: A rare case report
K Jayaprakash Shetty1, HL Kishan Prasad1, Sandeep Rai2, Y Sunil Kumar1, Shubha Bhat1, Netra Sajjan1, Chandrika Rao1
1 Department of Pathology, K S Hegde Medical Academy of Nitte University, Mangalore, Karnataka, India
2 Department of Paediatric Surgery, K S Hegde Medical Academy of Nitte University, Mangalore, Karnataka, India
|Date of Web Publication||9-Oct-2015|
H L Kishan Prasad
Department of Pathology, K S Hegde Medical Academy of Nitte University, Deralakatte, Mangalore, Karnataka
Source of Support: None, Conflict of Interest: None
Cervical region teratoma is a rare disease, accounting for 3-5% of all teratomas in the children. Teratomas of the head and neck due to their obscure origin, unpredictable behavior, and often manifest as a clinical surprise. Airway obstruction is the most serious postnatal complication of cervical teratoma. Prenatal diagnosis is crucial for early recognition of the neck masses that could obstruct the airway. We present a case of 4-month-old female child at age of 4 th month with right submandibular region swelling. Computed tomography neck showed ill-defined, multiloculated cystic lesion with enhancing thick septations in the right side of the neck. Excision biopsy revealed Grade I - immature teratoma - cervical region. On 1-year of close follow-up, no evidence of local recurrence or metastasis was seen. Unlike adults, teratomas in children are often congenital and very rarely turn malignant. The treating consultant should be aware of their natural history, clinical features, pathology, and principles of management.
Keywords: Cervico teratoma, child, immature teratoma, paediatric teratoma, teratoma
|How to cite this article:|
Shetty K J, Kishan Prasad H L, Rai S, Kumar Y S, Bhat S, Sajjan N, Rao C. Unusual presentation of immature teratoma of the neck: A rare case report. J Can Res Ther 2015;11:647
|How to cite this URL:|
Shetty K J, Kishan Prasad H L, Rai S, Kumar Y S, Bhat S, Sajjan N, Rao C. Unusual presentation of immature teratoma of the neck: A rare case report. J Can Res Ther [serial online] 2015 [cited 2020 Jun 4];11:647. Available from: http://www.cancerjournal.net/text.asp?2015/11/3/647/137994
| > Introduction|| |
Teratomas are complex tumors composed of a collection of heterogeneous cells and/or organoid structures reminiscent of normal derivatives of all three germ cells. ,, They occurs in genital systems and other organs along the midline of the body, with similar morphology. , Ovarian teratomas, constitute the major fraction and make up 15-20% of all ovarian tumors. ,, Teratomas of the neck are interesting because of their obscure origin, bizarre microscopic appearance, unpredictable behavior, and often-dramatic clinical presentation. , Cervical region teratomas constitutes of 3% of all teratomas.  In children, they are usually diagnosed early due to the frequent symptoms such as respiratory distress, facial disfigurement and orbital involvement. Unlike adults, teratomas in children are often congenital and very rarely turn malignant. ,, The treating clinician should be aware of their natural history, clinical features, pathology, and principles of management. This article focuses on the diversity and rarity of this group of tumors.
| > Case report|| |
A 4-month-old female child presented with neck swelling since birth. Swelling was gradually progressive. Local examination revealed, firm and nontender, 5 cm × 4 cm swelling in the right submandibular region. Skin over the swelling was unremarkable. Family and developmental history was unremarkable. Routine hematological, biochemical, and microbiological examinations were unremarkable. Computed tomography neck showed ill-defined, multiloculated cystic lesion with enhancing thick septations in the right side of the neck [Figure 1], pushing the right parotid superiorly with maintained surrounding fat planes, few calcific specks, and solid areas in the anteroinferior aspect with multiple enlarged bilateral cervical lymph nodes. Differential diagnosis considered was infected cystic hygroma or teratoma. Fine-needle aspiration cytology of the lesion was inconclusive with few scattered lymphocytes. Hence, complete excision was performed. Lesion was approached with right cervical transverse incision. The tumor was well-encapsulated, moderately vascular and showed adhesion with right lobe of the thyroid. Lesion was dissected out completely maintaining good hemostasis. Grossly, the mass was measuring 5 cm × 4 cm with grey brown and nodular areas. Focally cystic change and mucoid areas were seen. Histopathology showed tumor with components of all three germ cell layers comprising cartilage, glial tissue [Figure 2] and [Figure 3], choroid plexus, and skin with adnexal structures, respiratory mucosa, thyroid tissue, and fatty tissue. Focally immature neuroepithelial tissue having small round cells centered around the neurofibrillar material [Figure 4] and [Figure 5]. Features were consistent with Grade 1 immature teratoma-neck. Resected margins were free from tumor. Serum alpha-fetoprotein was 28.8 IU/ml. On 1-year of close follow-up, no evidence of local recurrence or metastasis was seen.
|Figure 1: Computed tomography neck showing ill-defined, multiloculated cystic lesion with enhancing thick septations in the right side of neck|
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|Figure 2: Histopathology showing glial and choroidal tissue (H and E, ×100)|
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|Figure 3: Histopathology showing respiratory epithelium with subepithelial cartilage (H and E, ×100)|
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|Figure 4: Histopathology showing immature neuroepithelial with rosettes (H and E, ×100)|
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|Figure 5: Histopathology showing rosettes with central fibrillar material (H and E, ×400)|
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| > Discussion|| |
The term "neck teratoma" generally incorporates lesions arising in the anterior and posterior triangles of the neck and excludes those arising from the base of the skull or from the spine. , Sub classification of cervical teratomas into thyroid teratomas, cervical, or extrathyroid teratomas, based on their blood supply, have not been acceptable. , Moreover, as in our case and most cases in the literature it is difficult to determine the origin of the main feeding vessels. In the literature, most of the teratomas originating anteriorly in the neck had some relation and connection to the thyroid. ,, The tumor in our case was partly adherent to the thyroid gland. This relationship to the thyroid gland was so constant that Roediger and associates have argued that all teratomas presenting in the anterior neck region arise from embryonic cells in the primitive analogue of the thyroid gland. , In the rare instances in which muscle and soft tissue are interposed, this may be due to mechanical dislocation of embryonic teratogenic cells in a manner analogous to the dislocation of normal thyroid tissue that comes to lie adjacent to, and at some distance from the normal gland. , Most of the cases in the literature present clinically as a large oval mass in the neck and the consistency of the mass usually appears partly cystic and partly solid. ,, There is no predilection for left, right, or median regions of the neck.
All races and both sexes are affected with equal frequency. The largest tumor reported in the literature had extended to the mastoid process superiorly and to the clavicles inferiorly. ,, Most neck teratomas are reported as mature, and malignant immature teratomas have been said to occur in approximately 5% of teratomas of the neck. ,,
The prognosis of neck teratomas is poor without surgical intervention. Hence, unless surgery is instituted without delay, the prognosis of cervical teratoma can be serious. ,,, This grave outlook seems to depend largely upon obstruction of upper respiratory passages. , Tracheostomy is difficult without removal of the tumor in patients presenting with very large masses. ,, In prenatal life, this causes polyhydramnios; postnatally, it may lead to aspiration of secretions, atelectasis, and bronchopneumonia. ,,, The operative mortality has been reported at between 9% and 17%, respectively. Removal of the tumor is the treatment of choice for this condition. There is no evidence that radiation is beneficial, and it carries potential risks. Further studies are required to define the proper line of management and therapy for these patients. ,,
Malignant transformation of benign teratoma in children is possible, but the malignant teratoma can be curable with multi-agent chemotherapy along with complete resection. The malignant component can be monitored by serial alpha-fetoprotein (AFP) measurements and complete resection is assured by the decline of AFP to normal after cessation of the chemotherapy. Multi-disciplinary management for the disease should be continued from neonatal period until growth has finished. ,,
| > Conclusion|| |
Cervical region teratomas in neonates are mostly benign, but their perinatal management and treatment strategy is challenging. A multidisciplinary team should be involved for securing the airway and complete excision to reduce the risk of malignant change. Surgical resection is done after the precise tumor localization-using multimodal imaging techniques to conserve the functional and cosmetic outcome.
| > References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]