Mandibular metastasis as a presenting feature of a clival chordoma

Chordomas are rare tumors which arise from the embryological remnants of the notochord. These tumors can potentially arise from any region within the craniospinal axis and often clinically present as a diagnostic challenge. Chordomas are rare in patients younger than 40 years of age. The most common primary cancers that metastasize to the jaw bones are the ones originating from the breast, lung, kidney adrenal, colo-rectum, or prostate. Mandibular metastasis from a primary chordoma is an extremely rare occurrence with only five prior reports, three originating from primaries in the sacrococcygeal region, one from a lumbar spine primary and the other from a primary arising from the spheno-occipital region. A literature review did not reveal any prior reports of mandibular metastasis at presentation from a clival chordoma. We possibly report the first case of such an unusual clinical scenario in a 7-year-old male child and further discuss the evaluation and management of these rare tumors

Neoadjuvant concurrent chemoradiation in male breast cancer: Experience from a tertiary cancer center

BACKGROUND: Male breast cancers (MBC) account for 1% of all breast cancers. Neoadjuvant concurrent chemoradiation (CTRT) is not the standard of care for treating breast cancer. However, in our center, it has been routinely used in patients with locally advanced breast cancer to downsize the tumor and make it amenable to surgery. AIM: This study was conducted to examine the clinical and pathological profile and outcomes of patients with MBC treated at our institute with neoadjuvant CTRT. SETTINGS AND DESIGN: The study was conducted at a tertiary cancer center and was retrospective in nature. MATERIALS AND METHODS: All MBC patients treated with neoadjuvant CTRT at our center between 2001 and 2016 were enrolled in the study. Data were retrospectively extracted from the patients' case records. STATISTICAL ANALYSIS: Kaplan–Meier method was used for survival analysis and the outcome variables were compared using the log-rank test. RESULTS: Thirty-one MBC patients who received neoadjuvant CTRT were analyzed in this study. The median age of the patients was 53 years. Stage IIB disease was observed in 8/31 (26%) patients, stage III in 20/31 (64%), and stage IV in 3/31 (10%) patients. There was no grade 3 or 4 toxicity due to CTRT. Surgery was performed in 29/31 (94%) patients and none of the patients had a pathological complete response. The median duration of follow-up was 95.3 months. The 8-year event-free survival and overall survival for stage IIB, III, and IV were 75%, 50%, and 0% and 87.5%, 69%, and 0%, respectively. CONCLUSION: This is the first study to report on the use of neoadjuvant CTRT in MBC. Prospective evidence from phase-3 randomized controlled trials on the safety and efficacy of CTRT in breast cancer is required before its routine use can be recommended.

Management outcomes of pediatric and adolescent papillary thyroid cancers with a brief review of literature

Background: Papillary carcinoma of thyroid (PTC) is a rare disease in children and adolescents and contributes to about 1.5%–3% of all pediatric malignancies. To date, no randomized trial has ever been performed in the pediatric population and management of these patients has been extrapolated from adult practice. Materials and Methods: Retrospective analysis of the patients treated for PTC in the age <21 years, between the years 1998–2013 at a tertiary cancer center from India. Results: Sixty-seven patients were treated in the above said period with a male:female ratio of 1:1.6 and a median age of 18 years. Fifty-two (77.6%) patients clinically presented as a thyroid swelling with or without nodal swelling while 13 (19.4%) presented with isolated nodal swelling. Surgery was performed in 30 patients at a nononcological hospital and was subsequently referred to our center; more than half of them needed a completion surgery at our center. Pathologically, multifocal tumors were found in close to a quarter of the patients. Among the pathological variants, classical, follicular, and tall cell variants comprised 65.7%, 28.4%, and 5.9% of the cases, respectively. Nodal positivity was noted 71.6% of the cases of which 14.5% were N1a disease and the vast majority (85.5%) harboring N1b disease. The median follow-up period of the study cohort was 104 months during which there were 3 local, 6 nodal, and 2 systemic recurrences. The 5- and 10-year disease-free survival were found to be 85.9% and 81.4%, respectively. Univariate and multivariate analysis has shown no significant clinical and pathological feature defining the disease outcomes except for the T-stage. Logistic regression revealed extrathyroidal invasion and the age ≤ 15 years correlated with nodal positivity. Conclusion: Being a rare malignancy, pediatric and adolescent PTCs tend to behave differently from adult PTC with a seemingly aggressive clinical presentation; however, they are associated with excellent survival outcomes.

Cytokeratin-positive interstitial reticulum cell sarcoma: Extranodal presentations mimicking carcinoma.

Cytokeratin-positive interstitial reticulum cell (CIRC) sarcoma is a rare type of dendritic cell tumor derived from a subset of fi broblastic reticular cells. Expression of cytokeratins and extranodal location of these tumors can lead to a misdiagnosis of carcinoma. We report two cases of CIRC sarcomas primarily involving the scalp and breast. Patients were referred with an initial diagnosis of carcinoma. Case 1 underwent wide local excision of the scalp tumor with left posterolateral neck dissection. Case 2 had modifi ed radical mastectomy for the tumor in left breast. Histopathological examination of both specimens showed an epithelioid to spindle cell malignant tumor that co-expressed CK 8, CK 18, vimentin, and smooth muscle actin. A diagnosis of CIRC sarcoma was made. Pathologists should be aware of this subset of dendritic cell sarcoma. Carcinomas, other sarcomas of the accessory dendritic cell family, and poorly differentiated malignant tumors have to be ruled out by combination of morphology, immunohistochemistry, and electron microscopic studies.