ABSTRACT
BACKGROUND: Müllerian remnant leiomyomas occur in women with Mayer-Rokitansky-Küster Hauser syndrome in which leiomyomas arise in absence of a uterus. This is a rare condition and we present two unusual cases. CASES: In the first case, a woman with Mayer-Rokitansky-Küster-Hauser syndrome diagnosed was found to have a pelvic mass and was scheduled for laparotomy. A few days before the surgery, acute abdominal pain developed. At surgery she was found to have a twisted adnexum including a Müllerian remnant leiomyoma. This was untwisted successfully; the leiomyoma was excised and the ovary was saved. In the second case, one member of a pair of monozygotic twins was found to have leiomyomas at age 33 after having an absent uterus diagnosed at laparoscopy 8 years previously. She had successful removal of the leiomyoma at laparotomy. Her sister had normal development and had two children. CONCLUSION: Mayer-Rokitansky-Küster-Hauser syndrome leading to Müllerian remnant leiomyomas is uncommon but should be suspected in women in whom a pelvic mass develops after the confirmation of the Müllerian defect.
Subject(s)
46, XX Disorders of Sex Development/complications , Adnexal Diseases/etiology , Leiomyoma/complications , Mullerian Ducts/pathology , Torsion Abnormality/etiology , Abnormalities, Multiple , Adnexal Diseases/surgery , Adult , Congenital Abnormalities , Female , Humans , Kidney/abnormalities , Leiomyoma/surgery , Somites/abnormalities , Spine/abnormalities , Torsion Abnormality/surgery , Twins, Monozygotic , Uterus/abnormalities , Vagina/abnormalitiesABSTRACT
Squamous cell carcinoma is a rare thyroid neoplasm that has been described exclusively in adults. We report what appears to be the first example of a primary squamous cell carcinoma of the thyroid gland arising in a background of Hashimoto's thyroiditis in an adolescent female. The tumor was well demarcated, confined to the right thyroid lobe, and did not metastasize, although follow up has been limited. The squamous cell carcinoma was well to moderately differentiated, and the stroma contained an abundant inflammatory infiltrate rich in lymphocytes and eosinophils. The lack of goblet cells, extracellular mucin, and extensive stromal sclerosis excluded the diagnosis of sclerosing mucoepidermoid carcinoma with eosinophilia. Immunohistochemical staining revealed focal expression of cytokeratin 7 and diffuse labeling with cytokeratin AE1/AE3. The squamous cell carcinoma overexpressed p53 protein and showed increased proliferative activity, as evidenced by the high MIB-1 labeling index. In contrast, the tumor did not show immunoreactivity for thyroglobulin or thyroid transcription factor 1.