scholarly journals Conjunctival Leiomyosarcoma, a Rare Neoplasm Always Originating at the Limbus? Report of a New Case and Review of 11 Published Cases

2019 ◽  
Vol 5 (5) ◽  
pp. 333-339 ◽  
Author(s):  
V. De Groot ◽  
E. Verhelst ◽  
P.C.W. Hogendoorn ◽  
R.J.W. de Keizer
Keyword(s):  
Adjuvant Radiotherapy ◽  
Myogenic Differentiation ◽  
Distant Metastases ◽  
Excisional Biopsy ◽  
Complete Resection ◽  
Favorable Prognosis ◽  
Strontium 90 ◽  
Malignant Mesenchymal Tumor ◽  
Histopathological Investigation

Purpose of the Study: To describe the differential diagnosis and management of a rare conjunctival malignancy. Procedures: A 79-year-old man presented with a conjunctival mass at the limbus. Excisional biopsy revealed a malignant mesenchymal tumor with myogenic differentiation. Six months later, three suspect lesions developed at the border of the previous excision. Pathological diagnosis pointed to a leiomyosarcoma. Adjuvant radiotherapy with strontium-90 brachytherapy was applied. After 3 years, there was neither recurrence nor distant metastases. A literature review revealed 11 cases of conjunctival leiomyosarcoma. Results: All 12 cases seemed to originate at the limbal conjunctiva. Scleral invasion was found only in one patient with multiple previous resections. Corneal invasion was reported in two patients. Four patients had a globe-sparing resection. In two of them, margins were not tumor free and additional brachytherapy gave a tumor-free follow-up of 1 and 3 years. Four cases underwent an exenteration. Conclusion and Message: Primary conjunctival leiomyosarcoma is a rare tumor with a favorable prognosis due to early detection and consequently limited size. Diagnosis involves histopathological investigation including immunohistochemistry. If possible, complete resection has the best prognosis. Adjunctive radiotherapy can be effective when the margins are not free and should be considered.

scholarly journals The radiation therapy options of intracranial hemangiopericytoma: An overview and update on a rare vascular mesenchymal tumor

2018 ◽  
Author(s):  
Maria Paola Ciliberti ◽  
Rosa D'Agostino ◽  
Laura Gabrieli ◽  
Anna Nikolaou ◽  
Angela Sardaro
Keyword(s):  
Surgical Resection ◽  
Adjuvant Radiotherapy ◽  
Therapeutic Option ◽  
Treatment Options ◽  
Distant Metastases ◽  
Complete Resection ◽  
Complete Surgical Resection ◽  
Hypervascular Tumor ◽  
The Central Nervous System ◽  
Radiotherapy Treatment

Hemangiopericytoma (HPC) is an extremely rare hypervascular tumor of mesenchymal lineage. It tends to recur and to develop distant metastases even many years after primary surgical resection. The management of recurrent and metastatic disease is not always so well defined. A complete surgical resection does not eliminate the high risk of local recurrences that occur in the central nervous system, often in the same surgical bed. However, treatment with adjuvant radiotherapy even in cases of complete resection remains controversial. Because of its rarity, there is no standard for treatment. We focused on radiotherapy treatment options, analyzing the literature and making a base on conduct further studies to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease and to help specialists to take the most appropriate therapeutic option.

Intraductal carcinoma of the breast: results of treatment with excisional biopsy and irradiation.

1985 ◽  
Vol 3 (10) ◽  
pp. 1339-1343 ◽  
Author(s):  
A Recht ◽  
B S Danoff ◽  
L J Solin ◽  
S Schnitt ◽  
J Connolly ◽  
...  
Keyword(s):  
Nipple Discharge ◽  
Distant Metastases ◽  
Excisional Biopsy ◽  
Intraductal Carcinoma ◽  
Carcinoma Of The Breast ◽  
Palpable Mass ◽  
Results Of Treatment ◽  
Number Of Patients ◽  
Boost Dose

Between 1976 and 1983, 40 women with intraductal carcinoma of the breast without invasion underwent excisional biopsy and irradiation as an alternative to mastectomy. The median age was 53 years (range, 28 to 77 years) and the median follow-up time since initiation of radiation was 44 months (range, 14 to 97 months). Twenty-seven patients presented with a palpable mass; in 13 patients the tumor was detected only by mammography. A limited axillary dissection was performed in 13 patients, and all lymph nodes removed were negative. Treatment was administered to the breast and adjacent chest wall to a dose of 4,600 to 5,000 rad, with 26 patients also receiving a boost dose of 1,000 to 2,000 rad to the site of the primary. Four patients have developed a recurrence in the treated breast, at 17, 19, 35, and 63 months after the beginning of radiation therapy. The 5-year actuarial rate of local recurrence is 10%. Three of the recurrences were in those four patients who presented with a nipple discharge and a central primary. In two cases, the recurrence consisted of only intraductal carcinoma; in the other two, both intraductal and invasive cancer were found. All four patients with recurrence underwent mastectomy and are well without evidence of distant metastases at 1, 12, 15, and 15 months since mastectomy. Cosmetic results were excellent. No patient has developed distant metastases. Since the number of patients treated is small and the period of follow-up is short, one must be cautious in the interpretation of these results. Nonetheless, the treatment of intraductal carcinoma of the breast by excision and irradiation appears to give acceptable local control and excellent survival when suitable precautions of patient selection and evaluation are taken.

scholarly journals Alveolar soft part sarcoma of the orbit, a case report of a rare tumor

2017 ◽  
Vol 9 (1) ◽  
pp. 70-73 ◽  
Author(s):  
Ángel Nava-Castañeda ◽  
José Luis Tovilla-Canales ◽  
Francisca Zuazo ◽  
Lourdes Rodríguez-Cabrera ◽  
Lilia Garnica-Hayashi
Keyword(s):  
Malignant Tumor ◽  
Soft Part ◽  
Extended Period ◽  
Distant Metastases ◽  
Excisional Biopsy ◽  
Lower Eyelid ◽  
Soft Tissue Tumors ◽  
Alveolar Soft Part Sarcoma ◽  
The Right

Background: Alveolar Soft Part Sarcoma is a rare malignant tumor of uncertain histogenesis, representing 0.5-1% of all soft tissue tumors. It occurs predominantly in head and neck regions, especially the orbit and the tongue, in infants, causing no specific symptoms for an extended period of time. Case: A four year old girl was brought to consultation because of persistent swelling of her left upper and lower eyelid for the past six months. A 4 mm proptosis was noted on her left eye with significant upwards displacement of the globe. A firm, not pulsating and non tender mass was palpated, and motility examination revealed deficient abduction and infraduction. Visual acuity was 20/40 and 1/200 in the right and left eye, respectively. The computed tomography revealed an extraconal mass on the inferotemporal aspect of the left orbit, with no bony erosion or globe invasion. An excisional biopsy was made, finding a tumor with nests of clusters of large polygonal cells, separated by fibrous septa and a sinusoidal vascular channel. An Alveolar Soft Part Sarcoma was diagnosed. Our patient had a favorable post-operative follow up, and oncologic evaluations have not shown metastasis or local recurrences. Observations and Conclusions: Alveolar Soft Part Sarcoma is a rare malignant tumor of indolent course, but with propensity to distant metastases, making early diagnose and long term follow up necessary. 

scholarly journals Tracheal Chondrosarcoma: Systematic Review of Tumor Characteristics, Diagnosis, and Treatment Outcomes with Case Report

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Emily A. Kutzner ◽  
Joshua S. Park ◽  
Salman Zaheer ◽  
Jared C. Inman
Keyword(s):  
Systematic Review ◽  
Treatment Outcomes ◽  
Adjuvant Radiotherapy ◽  
Complete Resection ◽  
Definitive Treatment ◽  
Tracheal Resection ◽  
Common Symptom ◽  
Tumor Characteristics ◽  
Outcomes Management

To our knowledge this is the first systematic review of tracheal chondrosarcoma treatment outcomes. Management insights are thoroughly discussed. Men constitute 93.8% of cases, and most of these occur in the distal trachea. The most common symptom, dyspnea, occurs in virtually all patients. Extratracheal extension had occurred in 78.6% of patients. Definitive treatment with tracheal resection showed no recurrences in 10 patients with mean follow-up of 3.1 years. Adjuvant radiotherapy may be utilized for improving local control when open complete resection cannot be performed, but only after endoscopic excision of gross tumor.

scholarly journals The Use of Strontium-90 Beta Radiotherapy as Adjuvant Treatment for Conjunctival Melanoma

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Victoria M. L. Cohen ◽  
Vasilios P. Papastefanou ◽  
S. Liu ◽  
Ian Stoker ◽  
John L. Hungerford
Keyword(s):  
Visual Acuity ◽  
Adjuvant Treatment ◽  
De Novo ◽  
Excisional Biopsy ◽  
Bulbar Conjunctiva ◽  
Conjunctival Melanoma ◽  
Strontium 90 ◽  
Eye Symptoms ◽  
Dry Eye Symptoms

Background/Aims. To report the safety and efficacy of strontium (Sr90) beta radiotherapy as adjuvant treatment for conjunctival melanoma.Methods. A retrospective cohort study was undertaken from 1999 to 2007 of all patients who underwent Sr90beta radiotherapy for incompletely excised conjunctival melanoma. Failure of treatment was defined as recurrence of a conjunctival melanoma at the same location following beta radiotherapy.Results. Twenty patients underwent Sr90beta radiotherapy for incompletely excised conjunctival melanoma. Median follow-up interval was 59 months (8–152). All patients had conjunctival melanoma involving the bulbar conjunctiva. Underlying diagnoses included PAM with atypia in 60% (12 of 20), PAM without atypia in 15% (3 of 20), and de novo conjunctival melanoma in 25% (5 of 20). Following Sr90beta radiotherapy, in 90% (18 out of 20) local control was achieved and visual acuity was not affected in any patient. Three patients (15%) had dry eye symptoms, episcleritis, and descemetcoele, respectively. No cataract or secondary glaucoma was reported.Conclusions. Sr90treatment is a very effective adjuvant treatment after excisional biopsy and cryotherapy for conjunctival melanoma with a local success rate of 90%. The treatment is not associated with significant side effects and visual acuity is not affected.

Undifferentiated endometrial sarcomas (UES): Results of a French sarcoma group (FSG) retrospective series of 52 patients (pts).

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e17109-e17109 ◽  
Author(s):  
Marie Meurer ◽  
Anne Floquet ◽  
Antoine Italiano ◽  
Morgane Auriche ◽  
Julien Mancini ◽  
...  
Keyword(s):  
Radical Hysterectomy ◽  
Adjuvant Radiotherapy ◽  
Univariate Analysis ◽  
Pelvic Mass ◽  
Figo Stage ◽  
Complete Resection ◽  
Significant Prognostic Factor ◽  
Curative Intent ◽  
Operative Strategy

e17109 Background: UES are rare tumors of very poor prognosis. Although large surgical resection is the cornerstone of curative intent treatment, the optimal post-operative strategy remains unclear. Methods: We conducted a retrospective analysis of UES pts over the last 8 years in 10 FSG centers, from Netsarc and RRePs databases. Results: Fifty-two pts with primary UES treated from 2008 to 2016 were included. The median age was 59 years (range, 42-82), and median ECOG was 0 (range, 0-3): 33 were localized (FIGO I-III) and 19 metastatic at diagnosis. Metrorrhagia, abdominal pain, and pelvic mass bleeding were the most common symptoms. Surgical procedures were radical hysterectomy and bilateral adnexectomy for 23/33 (70%) of localized and 11/19 (58%) of metastatic UES. Metastases were identified post operatively for 9/19 pts. In the localized group, 19 pts (58%) received radiotherapy, 10 (30%) complementary brachytherapy and 9 (27%) adjuvant chemotherapy. In the metastatic group, 16/19 (84%) received chemotherapy (polyCT, n=11). With a median follow-up of 28 months (0.3-112),24/52 pts are alive, 18/33 (58%) localized pts relapsed with 6 local relapses and 11 metastases. The 3- and 5-year Overall Survival (OS) rates were 40.7% and 29.1% respectively, for the whole group. In the localized group; median OS and Relapse-Free Survival (RFS) were 32.7 (23.3-42.1) and 22.9 months (2.3-43.7). In univariate analysis; early FIGO stage (p <0.0001), complete resection (R0-R1 vs R2; p=0.015) and adjuvant radiotherapy (p=0.001) were associated with a better OS; complete resection (p=0.021) and adjuvant radiotherapy (p=0.026) were associated with a better RFS. In the metastatic group, median OS was 16.4 months (12.0-20.7). In univariate analysis, no significant prognostic factor was retrieved neither on OS nor on RFS. Conclusions: Treatment of primary UES is radical hysterectomy and bilateral adnexectomy. Adjuvant radiotherapy appears beneficial for RFS. Due to the rarity and complexity of UES, referring patients to expert sarcoma centers is highly recommended.

scholarly journals CLINICAL AND MORPHOLOGICAL CHARACTERISTICS OF THE CASE OF I NTRA-PULMONARY METASTASES OF BENIGN LEIOMYOMA

2019 ◽  
Vol 25 (4) ◽  
pp. 81-86
Author(s):  
A. V. Reshetov ◽  
V. N. Klimenko ◽  
A. V. Elkin ◽  
L. B. Mitrofanova ◽  
I. V. Antonova ◽  
...  
Keyword(s):  
Morphological Characteristics ◽  
Distant Metastases ◽  
Excisional Biopsy ◽  
Subsequent Performance ◽  
Long Term Follow Up ◽  
Benign Leiomyoma ◽  
Antiestrogen Therapy ◽  
Immunohistochemical Studies

Uterine leiomyoma is rarely accompanied by the detection of distant metastases, including lungs. The basis of modern strategies of diagnosis of metastatic leiomyoma is the carrying out incisional or excisional biopsy of identified lesions with the subsequent performance of the immunohistochemical studies. Taking into account the indolent course of the disease, in modern practice, the main tactics of management of such patients is a long-term follow-up with the appointment of antiestrogen therapy.

Microsurgical resection of foramen magnum meningioma through a transcondylar approach: three-dimensional operative video

2019 ◽  
Vol 1 (2) ◽  
pp. V3
Author(s):  
Guilherme H. W. Ceccato ◽  
Rodolfo F. M. da Rocha ◽  
Duarte N. C. Cândido ◽  
Wladimir O. Melo ◽  
Marcio S. Rassi ◽  
...  
Keyword(s):  
Nerve Palsy ◽  
Three Dimensional ◽  
Foramen Magnum ◽  
Complete Resection ◽  
Neurophysiological Monitoring ◽  
Cervical Instability ◽  
Transcondylar Approach ◽  
Postoperative Mri ◽  
Microsurgical Resection

Foramen magnum (FM) meningiomas are challenging lesions. We present the case of a 38-year-old female with neck pain, dysphonia, and slight twelfth nerve palsy. Imaging workup was highly suggestive of an FM meningioma, and microsurgical resection with the aid of intraoperative neurophysiological monitoring was indicated. A transcondylar approach was employed, the vertebral artery was mobilized, and the tumor was completely removed. Postoperative MRI demonstrated complete resection. There were no signs of cervical instability. The patient presented with improvement of her symptoms and no new neurological deficit on follow-up. FM meningiomas can be successfully resected using a transcondylar approach, since it increases the exposure of the ventral FM, allowing the surgeon to work parallel to the skull base and flush with the tumor’s attachment. Informed consent was obtained from the patient for publication of this operative video.The video can be found here: https://youtu.be/itfUOB-6zM0.

scholarly journals Stereotactic radiosurgery for treating meningiomas eligible for complete resection

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Maximilian I. Ruge ◽  
Juman Tutunji ◽  
Daniel Rueß ◽  
Eren Celik ◽  
Christian Baues ◽  
...  
Keyword(s):  
Adverse Events ◽  
Stereotactic Radiosurgery ◽  
Medical Condition ◽  
Local Tumor Control ◽  
Complete Resection ◽  
Neurological Deficits ◽  
Tumor Control ◽  
Recurrence Rates ◽  
Treatment Volume

Abstract Background For meningiomas, complete resection is recommended as first-line treatment while stereotactic radiosurgery (SRS) is established for meningiomas of smaller size considered inoperable. If the patient´s medical condition or preference excludes surgery, SRS remains a treatment option. We evaluated the efficacy and safety of SRS in a cohort comprising these cases. Methods In this retrospective single-centre analysis we included patients receiving single fraction SRS either by modified LINAC or robotic guidance by Cyberknife for potentially resectable intracranial meningiomas. Treatment-related adverse events as well as local and regional control rates were determined from follow-up imaging and estimated by the Kaplan–Meier method. Results We analyzed 188 patients with 218 meningiomas. The median radiological, and clinical follow-up periods were 51.4 (6.2–289.6) and 55.8 (6.2–300.9) months. The median tumor volume was 4.2 ml (0.1–22), and the mean marginal radiation dose was 13.0 ± 3.1 Gy, with reference to the 80.0 ± 11.2% isodose level. Local recurrence was observed in one case (0.5%) after 239 months. The estimated 2-, 5-, 10- and 15-year regional recurrence rates were 1.5%, 3.0%, 6.6% and 6.6%, respectively. Early adverse events (≤ 6 months after SRS) occurred in 11.2% (CTCEA grade 1–2) and resolved during follow-up in 7.4% of patients, while late adverse events were documented in 14.4% (grade 1–2; one case grade 3). Adverse effects (early and late) were associated with the presence of symptoms or neurological deficits prior to SRS (p < 0.03) and correlated with the treatment volume (p < 0.02). Conclusion In this analysis SRS appears to be an effective treatment for patients with meningiomas eligible for complete resection and provides reliable long-term local tumor control with low rates of mild morbidity.

scholarly journals Improved risk estimation of locoregional recurrence, secondary contralateral tumors and distant metastases in early breast cancer: the INFLUENCE 2.0 model

2021 ◽  
Author(s):  
Vinzenz Völkel ◽  
Tom A. Hueting ◽  
Teresa Draeger ◽  
Marissa C. van Maaren ◽  
Linda de Munck ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Locoregional Recurrence ◽  
Clinical Decision Making ◽  
Metastatic Breast ◽  
Distant Metastases ◽  
Time Dependent ◽  
Individual Risk ◽  
Primary Tumors ◽  
Statistical Approaches

Abstract Purpose To extend the functionality of the existing INFLUENCE nomogram for locoregional recurrence (LRR) of breast cancer toward the prediction of secondary primary tumors (SP) and distant metastases (DM) using updated follow-up data and the best suitable statistical approaches. Methods Data on women diagnosed with non-metastatic invasive breast cancer were derived from the Netherlands Cancer Registry (n = 13,494). To provide flexible time-dependent individual risk predictions for LRR, SP, and DM, three statistical approaches were assessed; a Cox proportional hazard approach (COX), a parametric spline approach (PAR), and a random survival forest (RSF). These approaches were evaluated on their discrimination using the Area Under the Curve (AUC) statistic and on calibration using the Integrated Calibration Index (ICI). To correct for optimism, the performance measures were assessed by drawing 200 bootstrap samples. Results Age, tumor grade, pT, pN, multifocality, type of surgery, hormonal receptor status, HER2-status, and adjuvant therapy were included as predictors. While all three approaches showed adequate calibration, the RSF approach offers the best optimism-corrected 5-year AUC for LRR (0.75, 95%CI: 0.74–0.76) and SP (0.67, 95%CI: 0.65–0.68). For the prediction of DM, all three approaches showed equivalent discrimination (5-year AUC: 0.77–0.78), while COX seems to have an advantage concerning calibration (ICI < 0.01). Finally, an online calculator of INFLUENCE 2.0 was created. Conclusions INFLUENCE 2.0 is a flexible model to predict time-dependent individual risks of LRR, SP and DM at a 5-year scale; it can support clinical decision-making regarding personalized follow-up strategies for curatively treated non-metastatic breast cancer patients.

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