Struma ovarii (SO) is a rare form of ovarian tumor, which is defined by the presence of thyroid tissue comprising more than 50% of the overall tumor volume. The vast majority of the variants of SO are benign; however, malignant tumors have been reported in a small percentage of cases. An aggressive multimodality approach using ovarian cancer staging laparotomy, total thyroidectomy along with radioactive iodine-131 ablation, and thyroxin suppression therapy has been shown to safely treat malignant SO both its initial presentation as well as in the event of any subsequent recurrence with excellent efficacy and possibly better oncological outcomes. The rarity of the disease and the lack of evidence surrounding its management and prognosis continue to remain a challenge to the treating clinician. We present a unique case of malignant SO with an incidental synchronous association of follicular variant of papillary carcinoma of the cervical thyroid gland, this is possibly the second case reported in the English language literature.
Ichthyosis uteri is a rare condition in which the entire surface of the endometrium is replaced by stratified squamous epithelium. Though the condition often is considered as benign, anaplastic and dysplastic changes have been reported. We describe herein a rare case of low-grade squamous cell carcinoma of endometrium associated with extensive ichthyosis uteri with dysplasia. The cervix showed moderate to severe dysplastic changes while the right fallopian tube showed extensive squamous metaplasia with dysplastic changes. We conclude that squamous cell carcinoma could develop into pre-existing ichthyosis uteri. Clinical HistoryA 65-year-old multi-gravida presented with complaints of abdominal pain and postmenopausal bleeding per vagina for seven months. Her past medical history was insignificant with no history of tuberculosis, inflammatory conditions of the uterus or iatrogenically introduced substances in uterus. She had attained menopause about 15 years back.Gynecological examination revealed atrophic ectocervix flushed with vagina. The vagina appeared normal. Uterus was bulky of around 12 weeks and adnexae were unremarkable. Colposcopy showed schiller's unstained areas on anterior lip of cervix. Ultrasound abdomen showed endometrial thickness of 2.6 cms. and fluid in the endometrial cavity. Ectocervical biopsy showed strips of moderate to severe dysplastic stratified squamous epithelium. Endometrial curetting revealed strips of stratified squamous epithelium showing moderate dysplastic changes. No normal endometrium was seen. The pyometra was drained, following which the patient underwent type-II radical hysterectomy. The procedure was well tolerated and the postoperative period was uneventful. Pathologic findings MacroscopicThe hysterectomy specimen revealed thickened and widened endometrial cavity with gray white nodule in the sub-adjacent myometrium. The cervix showed no obvious growth. The cut-section of the right fallopian tube showed thickened mucosa. Both the ovaries did not show any gross abnormality. MicroscopicThe sections revealed entire endometrium replaced by stratified squamous epithelium showing areas of heavy
Chondroid syringoma (CS) represents the cutaneous counterpart of mixed tumor (pleomorphic adenoma) of salivary glands. The malignant counterpart of CS, termed as “malignant CS” is a malignant eccrine neoplasm which lacks distinctive clinical features, often delaying initial diagnosis. Unlike its benign counterpart which often localizes in the head and neck region, malignant CS most often encountered in the trunk and the extremities. We report a rare case of an aggressive malignant CS of the left pinna with cervical lymph node metastasis. Our patient, to the best of our knowledge, possibly is the first case of malignant CS of the pinna and the fourth to arise in the head and neck region. The diagnostic challenges with an added emphasis on the role of positron emission tomography-computed tomography in aiding the management of this rare tumor are discussed.
BackgroundHereditary cancers account for 5–10% of cancers. In this study BRCA1, BRCA2 and CHEK2*(1100delC) were analyzed for mutations in 91 HBOC/HBC/HOC families and early onset breast and early onset ovarian cancer cases.MethodsPCR-DHPLC was used for mutation screening followed by DNA sequencing for identification and confirmation of mutations. Kaplan-Meier survival probabilities were computed for five-year survival data on Breast and Ovarian cancer cases separately, and differences were tested using the Log-rank test.ResultsFifteen (16%) pathogenic mutations (12 in BRCA1 and 3 in BRCA2), of which six were novel BRCA1 mutations were identified. None of the cases showed CHEK2*1100delC mutation. Many reported polymorphisms in the exonic and intronic regions of BRCA1 and BRCA2 were also seen. The mutation status and the polymorphisms were analyzed for association with the clinico-pathological features like age, stage, grade, histology, disease status, survival (overall and disease free) and with prognostic molecular markers (ER, PR, c-erbB2 and p53).ConclusionThe stage of the disease at diagnosis was the only statistically significant (p < 0.0035) prognostic parameter. The mutation frequency and the polymorphisms were similar to reports on other ethnic populations. The lack of association between the clinico-pathological variables, mutation status and the disease status is likely to be due to the small numbers.
Endobiliary metastasis from colorectal cancer can mimic ICC, and IHC studies may be needed to differentiate the two. Identifying endobiliary metastasis can have therapeutic and prognostic implications.
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