| Abstract|| |
Mature cystic teratoma, also known as dermoid cyst, is the most common germ cell tumor of the ovary with a relative incidence of 20%. Rarely, dermoid cysts undergo malignant transformation (0.94%–2%). We report a case of mucoepidermoid carcinoma within a mature cystic teratoma in a 35-year-old female.
Keywords: Dermoid cyst, mature cystic teratoma, mucoepidermoid carcinoma, ovary
|How to cite this article:|
Momin YA, Kulkarni MP, Pandav AB, Sulhyan KR. Mucoepidermoid carcinoma in a mature cystic teratoma: A rare case report with review of literature. Indian J Pathol Microbiol 2017;60:117-8
|How to cite this URL:|
Momin YA, Kulkarni MP, Pandav AB, Sulhyan KR. Mucoepidermoid carcinoma in a mature cystic teratoma: A rare case report with review of literature. Indian J Pathol Microbiol [serial online] 2017 [cited 2017 May 26];60:117-8. Available from: http://www.ijpmonline.org/text.asp?2017/60/1/117/200038
| Introduction|| |
Dermoid-associated malignancies are rare. Most common malignant tumor arising in dermoid cyst is squamous cell carcinoma while uncommon tumors include malignant melanoma, sebaceous carcinoma, and adenocarcinoma.,,,,,,,, Although mucoepidermoid carcinoma (MEC) is the most common malignant tumor of salivary glands, it can occur uncommonly in tear gland, breast, thyroid, bronchopulmonary region, and thymus. However, MEC arising within dermoid cyst is very rare. Literature search revealed only two cases of MEC arising in mature cystic teratoma.,,,,,,,,
| Case Report|| |
A 35-year-old 3 para, with an unremarkable maternal history, presented with pain in the lower abdomen for 3 months. She had no history of joint pains or psoriasis. She was nonreactive, with a normal routine hematologic examination. Ultrasound examination showed a large pelvic mass suggesting left ovarian neoplasm. She underwent hysterectomy with bilateral salpingo-oophorectomy, along with the part of omentum.
Grossly, the left ovary was enlarged, nodular, solid-cystic and measured 16 cm × 14 cm × 9 cm (point value 2)., On cutting open, the cystic component showed tufts of hair along with Rokitansky tubercle and a predominant component of solid gray-white tumor [Figure 1]a. Histopathological examination showed complete replacement of ovary by the tumor. The fibrocollagenous cyst wall was lined by malignant squamous epithelium which was seen proliferating and infiltrating the deeper tissue. The cyst wall showed hair and mature adipose tissue as components of mature cystic teratoma [Figure 1]b. The solid areas showed tumor composed of three types of cells in varying proportion, viz., squamous cells, intermediate cells, and mucocytes. The squamous cells showed individual cell keratinization and few keratin pearls [Figure 1]c. Intermediate cells showed clear cytoplasm and centrally placed nuclei arranged in sheets or pseudoglandular pattern and mucocytes interspersed in between the two [Figure 1]d. Intracytoplasmic and intraluminal mucin was noted which was highlighted by mucicarmine stain. The intervening stroma showed tumor giant cells, foreign body type giant cells, foamy histiocytes, and diffuse infiltration by mononuclear cells. Mitotic count was 0–2/hpf (point value 3)., Extensive areas of tumor necrosis were evident (point value 3)., Parametrium and all eight lymph nodes dissected were free of tumor. Diagnosis offered was high-grade MEC (point value 8),, arising in a mature cystic teratoma.
|Figure 1: (a) Gross photograph showing ovary with a solid-cystic tumor. The cystic component shows tufts of hair and a solid gray-white component with microcysts and necrotic areas. (b) Microphotograph showing dermoid cyst with hair shafts (H and E, ×10). (c) Microphotograph showing squamous cells with individually keratinized cells in sheets and pseudoglandular pattern (H and E, ×10). (d) Microphotograph showing intermediate cells with clear cytoplasm arranged in glandular pattern with intraluminal mucin (H and E, ×10)|
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| Discussion|| |
MEC has been reported in association with acute myeloid leukemia, HIV, psoriasis, rheumatoid arthritis, and chemotherapy. Our case showed no such association.
Literature search of all cases showing malignant transformation in ovary showed the presence of dermoid cyst or endometriotic cyst.,,, In cases discussed by Terada, transition between squamous cell carcinoma and endometriosis was recognized, suggesting that squamous element arose from endometriotic epithelium. Primary MEC has been reported in the cervix and as metastasis from primary salivary gland origin., Our case showed neither evidence of endometriosis nor any evidence of primary carcinoma elsewhere.
Grossly, malignancy associated dermoid shows cystic mass with a mural component.,,,,, Histologic appearance of ovarian MEC resembles conventional MEC showing three types of cells intermingled in varying proportion. Our case showed similar morphologic picture. The closest differential diagnosis is adenosquamous carcinoma which has a worse prognosis than MEC.,,,,,,,, However, there is a fine line of differentiation between the two. No carcinoma in situ, limited pearls, lobular arrangement with intermingled glands, presence of intermediate cells, and mucocytes define MEC, whereas adenosquamous carcinoma harbors an in situ component, with numerous pearls, glands at lower portion of the tumor, absence of intermediate cells, and mucocytes. Our case showed a few pearls with the presence of intermediate cells and mucocytes. Although some authors have claimed absence of pearls to be defining feature of MEC, the WHO  has suggested that limited pearls can be seen as was seen in our case. Although neural invasion, necrosis, increased mitosis, and cellular anaplasia are uncommon features of MEC depending on their presence, MEC is graded accordingly, as sum value of the points scored., According to the WHO grading system, our case was graded as high as the tumor showed extensive areas of necrosis and increased mitosis. Reported cases suggest that the surgical management is similar to that of adenocarcinoma of the ovary, including total abdominal hysterectomy, bilateral salpingo-oophorectomy along with bilateral pelvic and para-aortic lymph node dissection. There is a lack of consensus regarding the adjuvant treatment. Clinical staging seems to be the determining factor. Tumors with Stage IA have favorable prognosis while those with Stage IC carry a poor prognosis.,,,, Blissfully, in our case, although high grade, the tumor was confined to ovary with no nodal metastasis; hence, surgical resection seemed to be the optimum treatment. She tolerated the procedure well and is fine with 2 years of follow-up.
| Conclusion|| |
Dermoid-associated malignancies not only pose a diagnostic challenge but also portend a bad prognosis. MEC is a very rare, dermoid-associated malignancy and has a worse prognosis than squamous cell carcinoma. Hence, a meticulous search is important to diagnose this entity. The closest differential diagnosis is adenosquamous carcinoma. As limited numbers of cases are reported in the literature, not only prognosis but also management of MEC has yet to be determined.
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Conflicts of interest
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Amitkumar Bapuso Pandav
Department of Pathology, Government Medical College, Miraj - 416 410, Maharashtra
Source of Support: None, Conflict of Interest: None