scholarly journals Congenital orbital teratoma: a case report with preservation of the globe and 18 years of follow-up

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Panagiotis A. Tsoutsanis ◽  
George C. Charonis

Abstract Background Congenital orbital teratomas are extremely rare, usually benign neoplasms, comprised of cells originating from all three germ cell layers. Clinically the tumor appears solid, most of the times is intraconal and presents as a rapidly growing mass leading to a massive unilateral axial proptosis, chemosis, exposure keratopathy, markedly distended eyelids and often, loss of vision. To prevent these complications, tumor excision usually involves enucleation or even orbital exenteration. Case presentation We report a case of a 1-day old infant who presented with dramatic proptosis at birth due to a true congenital orbital teratoma. We describe the clinical findings, the preoperative neuroimaging, the surgical management which included complete tumor resection with preservation of the globe to allow for optimal orbital growth, the histopathological evaluation, and the clinical course during 18 years of follow up. Conclusion Every effort to salvage the globe should be made to achieve the best possible orbito-facial development. Furthermore, the value of prompt surgical management with a less invasive transconjunctival globe sparing procedure can be appreciated in our case.

2021 ◽  
Vol 29 (2) ◽  
pp. 267-270
Author(s):  
Mehmet Akif Önalan ◽  
Ahmet Demirkaya ◽  
Kemal Behzatoglu ◽  
Ersin Erek

Cardiac leiomyosarcoma is an extremely rare tumor with a poor prognosis. An 18-year-old female patient was admitted to our clinic with a left atrial leiomyosarcoma extending to the right lower pulmonary veins. We performed complete tumor excision by the right anterolateral mini-thoracotomy approach using minimally invasive techniques. After pathological confirmation of the tumor, right lower lobectomy was performed with the same incision one week later to prevent recurrence. Although no tumor remnant was found in the lobectomy specimen, adjuvant chemotherapy was started. No recurrence was detected during the 12-month follow-up. In conclusion, the right submammarian minithoracotomy approach has the advantages of its less invasive nature and suitability for complete tumor resection with lobectomy.


2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 10556-10556
Author(s):  
J. Li ◽  
F. J. Gong ◽  
J. Li ◽  
W. A. Wu ◽  
L. Shen

10556 Background: Imatinib benefits the patients with metastatic GIST but whether it is effective in the adjuvant setting after complete tumor resection of primary GIST is questionable. Methods: Patients who had undergone complete tumor resection with intermediate or high risk of recurrence (define) were enrolled in a single-center non-randomized open contrast study. Patients had adjuvant therapy with imatinib (400mg once a day) for 3 years commencing within 12 weeks of tumor resection, or had follow-up alone. We performed c-kit and PDGFRA mutation analysis for patients with archival tumor samples. The primary objective was recurrence-free survival (RFS) and the second objective was correlation of gene mutation profile with efficacy of adjuvant therapy. Results: One hundred and five patients were enrolled: 56 patients in adjuvant therapy group and 49 patients in control group. Median follow-up was 30 months (range 12–62); Median treatment duration in adjuvant therapy group were 20 months (range 12–36); There was relatively higher 1-year and 2-year RFS in the adjuvant therapy group compared with the control group (100% vs 89.8%,94.4% vs 60.0%) (Log-RankP< 0.001). Hazard risk was 0.13 (95% CI 0.039–0.438, P= 0.001) in Cox proportional hazard regression model. For further sub-group analysis, Log-rank and Cox regression analysis showed that adjuvant therapy could significantly decrease recurrence risk in patients with high risk disease (define)(2-year RFS: 91.5% vs 46,2%, Log-Rank: P<0.001, HR:0.107 (95%CI 0.031–0.370), P<0.001). c-kit and PDGFRA mutation were analyzed in forty-nine patients. There was a better survival benefit from adjuvant therapy for patients with c-kit exon 11 mutation. Conclusions: Adjuvant therapy with imatinib can improve 1-year and 2-year recurrence-free survival for patients with an intermediate or high risk of recurrence after complete tumor resection. No significant financial relationships to disclose.


2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Christopher Hein ◽  
Barry Watkins ◽  
Lee M. Zuckerman

Primary sarcomas of the thumb metacarpal are rare malignant lesions. Surgical treatment involves amputation versus tumor resection with thumb reconstruction. If complete tumor resection is possible, thumb preservation may be considered, as the thumb is vital to hand function. Following tumor resection, previous reports have described graft reconstruction with fusion to the trapezium or scaphoid. We present two cases of sarcoma necessitating resection of the thumb metacarpal that were reconstructed with an arthrodesis of the proximal phalanx to the second metacarpal shaft. Arthrodesis to the second metacarpal allows robust bony contact for fusion as well as improved resting position of the thumb. At 2- and 4-year follow-up, both patients have a stable, pain-free thumb without evidence of local recurrence.


2020 ◽  
Vol 128 (12) ◽  
pp. 835-844
Author(s):  
Fernando Guerrero-Pérez ◽  
Agustina Pia Marengo ◽  
Noemi Vidal ◽  
Carles Villabona

Abstract Purpose and Methods Phenotype transformation in pituitary adenomas (PA) is a little known and unexpected clinical phenomenon. We describe two illustrative cases and performed a systematic review of cases reported in literature. Results Case 1: A 24-year-old woman underwent surgery because of Cushing’s disease. A complete tumor resection and hypercortisolism resolution was achieved. Two years later, tumor recurred but clinical and hormonal hypercortisolism were absent. Case 2: A 77-year-old woman underwent surgery due to acromegaly. A complete tumor resection and GH excess remission was achieved. Four years later, tumor recurred but clinical and hormonal acromegaly was ruled out. Search of literature: From 20 patients (including our cases), 75% were female with median age 45 (19) years. Ten patients (50%) had initially functioning PA: 8 switched to NFPA (5 ACTH-secreting PA, 2 prolactinomas and 1 acromegaly) and 2 exchanged to acromegaly from TSH-secreting PA and microprolactinoma. One patient developed a pituitary carcinoma from ACTH-secreting PA. Ten patients (50%) initially had NFPA; 9 developed Cushing’s disease (4 silent corticotroph adenomas, 4 null cell PA and 1 managed conservatively). One patient with silent somatotroph PA changed to acromegaly. Treatments before transformation were surgery (80%), radiotherapy (40%), pharmacological (40%) and in 2 patients switching happened without any treatment. Median follow-up until transformation was 72 months (range 12–276). Conclusion PA can change from functioning to (NF) non-functioning (vice versa) and even exchange their hormonal expression. Clinicians should be aware and a careful lifelong follow-up is mandatory to detect it.


2016 ◽  
Vol 34 (10) ◽  
pp. 1050-1056 ◽  
Author(s):  
Maciej Murawski ◽  
Víola B. Weeda ◽  
Rudolf Maibach ◽  
Bruce Morland ◽  
Derek J. Roebuck ◽  
...  

Introduction The aim of this article is to present an experience of two prospective studies from the International Childhood Liver Tumor Strategy Group (SIOPEL 2 [S2] and SIOPEL [S3]) trials and to evaluate whether modified platinum- and doxorubicin-based chemotherapy is capable of increasing tumor resectability and changing patient outcomes. Methods Between 1995 and 2006, 20 patients with hepatocellular carcinoma (HCC) were included in the S2 trial and 70 were included in the S3 trial. Eighty-five patients remained evaluable. Results Response to preoperative chemotherapy was observed in 29 of 72 patients (40%) who did not have primary surgery, whereas 13 patients underwent upfront surgery. Thirty-three patients had a delayed resection. Thirty-nine tumors never became resectable. Complete tumor resection was achieved in 34 patients (40%), including seven of those treated with liver transplantation (LTX). After a median follow-up period of 75 months, 63 patients (74%) had an event (a progression during treatment, a relapse after treatment, or death from any cause). Sixty patients died. Twenty-three of 46 patients (50%) who underwent tumor resection died. Eighteen of 27 patients (63%) with complete tumor resection (without LTX) and 20 of 34 patients (59%) with LTX survived. Only one of seven patients (14%) with microscopically involved margins survived. Overall survival at 5 years was 22%. Conclusion Survival in pediatric HCC is more likely when complete tumor resection can be achieved. Intensification of platinum agents in the S2 and S3 trials has not resulted in improved survival. New treatment approaches in pediatric HCC should be postulated.


Author(s):  
MAKOTO TAKEDA ◽  
Yoshinori Onuki ◽  
Kosuke Oishi ◽  
Osamu Kubota ◽  
Takashi Uchiyama ◽  
...  

Introduction Appendiceal mucinous neoplasms can involve peritoneal pseudomyxoma or invasion of adjacent organs. This report describes a rare case in which a giant appendiceal mucinous carcinoma expansively developed in the retroperitoneum without perforating the abdominal cavity. Case presentation The patient was a 55-year-old woman with no relevant history who was admitted to our hospital after imaging examinations revealed a retroperitoneal tumor. The clinical diagnosis was a retroperitoneal tumor involving the hedge mucin. The patient underwent right hemicolectomy and partial resection of the duodenum and right abdominal wall to ensure complete tumor resection. Histopathological findings suggested that the tumor was a mucinous carcinoma arising from the appendix. The postoperative course was uneventful. Although adjuvant chemotherapy was performed for 6 months, peritoneal recurrence developed 7 years and 4 months postoperatively. Nine years have passed after surgery and the patient is alive under receiving chemotherapy. Conclusion Detailed pathological examinations revealed that the tumor originated from the appendix. The characteristics of mucinous carcinoma contributed to the extensive growth of the tumor.


2016 ◽  
Vol 140 (4) ◽  
pp. 371-375 ◽  
Author(s):  
David Bakiratharajan ◽  
Bharat Rekhi

Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain differentiation, initially described by Enzinger and colleagues. Until now, nearly 300 such cases have been reported worldwide. The histogenesis of these tumors remains controversial. These tumors show characteristic imaging findings and exhibit a spectrum of histopathologic features, including classical and atypical subtypes. Local recurrences and, occasionally, distant metastases have also been reported. A complete tumor resection forms the preferred treatment modality for these tumors, along with follow-up, as these tumors have an uncertain malignant potential. Lately, certain “molecular signatures” underlying OFMTs have been described that can further aid in reaching an accurate diagnosis for these tumors and unraveling their pathogenesis. This article is a review of the clinical, radiologic, histopathologic, and molecular features of OFMTs.


2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi241-vi241
Author(s):  
Yonggao Mou ◽  
Al-Nahari Fuad ◽  
Hao Duan ◽  
Zhenqiang He ◽  
Guanhua Zhang ◽  
...  

Abstract The intraoperative tumor-free principles refer to the operation techniques that must be carried out to prevent the exfoliation and planting of tumor cells during the operation, so as to prevent local recurrence and distant metastasis. Following the intraoperative tumor-free principles has been valued in resection of extracranial solid tumors. However, the significance of intraoperative tumor-free principles for brain tumors is still unclear. We retrospectively analyzed 106 patients with primary glioblastoma who underwent resection following the intraoperative tumor-free principles from 2010 to 2016. By February 28, 2019, 11 (10.4%) patients were lost to follow-up. The median overall survival (OS) was 20.2 months, and the 1-, 3-, and 5-year survival rates were 71%, 30%, and 26%, respectively. For patients with complete tumor resection, the median OS was 24.8 months, and the 1-, 3-, and 5-year survival rates were 80%, 38%, and 36%, respectively. Patients who received postoperative Stupp regimen had a median OS of 74.1 months, and the 1-, 3-, and 5-year survival rates were 82%, 51%, and 51%, respectively. A total of 36 patients had complete tumor resection followed by Stupp regimen. Their 1-, 3-, and 5-year survival rates were 94%, 68%, and 68%, respectively. Median OS was not reached. The patient with the longest OS had survived for 108 months and was still alive. In summary, the OS of patients in this study was relatively longer than that reported in most previous literatures, which suggests that following the intraoperative tumor-free principles in resection of GBM can benefit patient survival.


2018 ◽  
Vol 11 (2) ◽  
pp. 341-346 ◽  
Author(s):  
Filipe Rocha Da Silva ◽  
Alan Vitor Jerônimo Lima ◽  
Erick Willian Rocha Pereira  Albuquerque ◽  
Carlos Augusto Moreira-Silva ◽  
Nicole Maués Flexa De Oliveira ◽  
...  

Retroperitoneal liposarcoma is a rare type of cancer. Relapse after surgery is frequent, and relapsing tumors tend to be more aggressive and less differentiated each episode, worsening the prognosis. This report describes the case of a 42-year-old female diagnosed with retroperitoneal liposarcoma after complete tumor resection. At the 3-month follow-up, another expansive lipomatous mass in the retroperitoneal area almost the same size as the previous one was detected. The patient underwent a new surgery, followed by first-line treatment with a gemcitabine- and docetaxel-based regimen for 8 cycles. Finally, the patient achieved complete tumor remission confirmed by CT after the end of the treatment proposed. Although recurrence is a well-known characteristic of this neoplasia, no other case with such a vast expansion of a new tumor shortly after complete resection was found in the literature.


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