Secretory Carcinoma in Children and Young Adults: A Case Series

2021 ◽  
pp. 109352662110469
Author(s):  
Caroline T Simon ◽  
Jonathan B McHugh ◽  
Raja Rabah ◽  
Amer Heider

Secretory carcinoma (SC), previously known as mammary analogue secretory carcinoma, is a rare salivary gland neoplasm that typically presents as a slow-growing painless lesion in the head and neck. SC occurs mainly in adults but has been described in children with the youngest reported patient diagnosed at five years of age. In children the gender distribution has been reported as female to male ratio of 1:1.2. SC is generally considered a low-grade malignancy with characteristic morphological features and immunological profile. SC also harbors ETV6-NTRK3 fusion (t(12;15)(p13:q25)). Surgical resection with or without lymph node dissection is the standard treatment, with generally favorable clinical outcomes. Here we present a single institution case series of six patients (ages 9-21) with SC and a review of the previously described pediatric cases. Our small series showed male predominance in pediatric patients with predominantly low-grade and stage tumors. All cases underwent complete surgical resections and when follow up is available there was no evidence of recurrences or metastases. To the best of our knowledge, this is the only SC case series comprised exclusively of pediatric and youth patients.

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii138-ii138
Author(s):  
Iyad Alnahhas ◽  
Appaji Rayi ◽  
Yasmeen Rauf ◽  
Shirley Ong ◽  
Pierre Giglio ◽  
...  

Abstract INTRODUCTION While advocacy for inmates with cancer has recently gained momentum, little is known about management of brain tumors in inmates. Delays in acknowledging or recognizing nonspecific initial symptoms can lead to delayed diagnosis and treatment. Inmates with cancer are reported to either be ignored or receive substandard care due in part to cost or logistics (American Civil Liberties Union; ASCO Post 2018). METHODS In this retrospective study, we identified inmates with gliomas seen in the Ohio State University Neuro-oncology Center between 1/1/2010-4/20/2019. RESULTS Twelve patients were identified. Median age at presentation was 39.5 years (range 28-62). Eleven patients were Caucasian and one was African American. Diagnoses included glioblastoma (GBM) (n=6), anaplastic astrocytoma (n=1), anaplastic oligodendroglioma (n=1), low-grade astrocytoma (n=3) and anaplastic pleomorphic xanthroastrocytoma (n=1). Patients were more likely to present early after seizures or focal neurologic deficits (9/12) than after headaches alone. Patients with GBM started RT 12-71 days after surgery (median 34.5). One patient’s post-RT MRI was delayed by a month and another with GBM had treatment held after 4 cycles of adjuvant temozolomide (TMZ) due to “incarceration issues”. For one patient who received adjuvant TMZ, the facility failed to communicate with the primary team throughout treatment. Two patients suffered significant nausea while on chemotherapy due to inability to obtain ondansetron in prison, or due to wrong timing. 7/12 (58%) patients were lost to follow-up for periods of 3-15 months during treatment. Three patients refused adjuvant treatment. CONCLUSIONS Although this is a small series, our results highlight the inequities and challenges faced by inmates with gliomas who are more likely to forego treatments or whose incarceration prevents them from keeping appropriate treatment and follow-up schedules. Additional studies are needed to define and address these deficiencies in the care of inmates with brain tumors and other cancers.


2021 ◽  
Vol 09 (02) ◽  
pp. E258-E262
Author(s):  
Christian Suchy ◽  
Moritz Berger ◽  
Ingo Steinbrück ◽  
Tsuneo Oyama ◽  
Naohisa Yahagi ◽  
...  

Abstract Background and study aims We previously reported a case series of our first 182 colorectal endoscopic submucosal dissections (ESDs). In the initial series, 155 ESDs had been technically feasible, with 137 en bloc resections and 97 en bloc resections with free margins (R0). Here, we present long-term follow-up data, with particular emphasis on cases where either en bloc resection was not achieved or en bloc resection resulted in positive margins (R1). Patients and methods Between September 2012 and October 2015, we performed 182 consecutive ESD procedures in 178 patients (median size 41.0 ± 17.4 mm; localization rectum vs. proximal rectum 63 vs. 119). Data on follow-up were obtained from our endoscopy database and from referring physicians. Results Of the initial cohort, 11 patients underwent surgery; follow-up data were available for 141 of the remaining 171 cases (82,5 %) with a median follow-up of 2.43 years (range 0.15–6.53). Recurrent adenoma was observed in 8 patients (n = 2 after margin positive en bloc ESD; n = 6 after fragmented resection). Recurrence rates were lower after en bloc resection, irrespective of involved margins (1.8 vs. 18,2 %; P < 0.01). All recurrences were low-grade adenomas and could be managed endoscopically. Conclusions The rate of recurrence is low after en bloc ESD, in particular if a one-piece resection can be achieved. Recurrence after fragmented resection is comparable to published data on piecemeal mucosal resection.


2019 ◽  
Vol 29 (3) ◽  
pp. 517-522
Author(s):  
Roberto Crosa ◽  
Alejandro M. Spiotta

Introduction. Acute ischemic stroke due to large vessel occlusion refractory to aspiration or mechanical thrombectomy is a therapeuticchallenge. Objective. A treatment variant is in order. Methods. In the last three years, we admitted seven patients with refractory largevessel occlusions, for all of whose aspiration and mechanical thrombectomy had failed. A Solitaire AB stent was deployed as a rescuemeasure. Results. Data was retrospectively analyzed. Six out of seven patients had a good clinical outcome as measured by mTICIand mRS twelve months after the procedure. One patient died after the first follow-up visit, one month after endovascular therapy.Conclusion. Results of this small series support the role of stent deployment as a rescue measure for such challenging patients.


Author(s):  
Shashikant Anil Pol ◽  
Surinder K. Singhal ◽  
Nitin Gupta ◽  
Himanshu Bayad

<p>Mammary analogue secretary carcinoma are salivary gland tumors similar to secretary carcinoma of breast histologically. It usually affects adults with mean age group of 45 years. Clinically it is usually slow growing, low grade malignancy with favorable prognosis. We are reporting a case of 47 years old female presented with complaints of swelling behind left ear for last 7 years. It was slow growing, painless and persistent. On examination, 4 x 3 cm swelling was present just below left ear lobule. It was nontender, firm, irregular in shape with smooth surface with slight mobility and overlying skin pinchable. Fine needle aspiration cytology suggested benign tumor with cystic change. On contrast enhanced computed tomography scan, there was a lobulated hypodense lesion measuring 24×35×32 mm with internal septations and enhancement of wall in expected location of superficial as well as deep lobe of left parotid gland. Patient underwent left total conservative parotidectomy and histopathology came out to be secretory analogue mammary carcinoma. Subsequently she received post-operative radiotherapy. Patient is on regular follow up and disease free till date. Usually mammary analogue secretory carcinoma is a low-grade malignancy with good prognosis. Immunohistochemistry is confirmatory which shows positivity for S-100 protein and mammaglobin.</p>


2011 ◽  
Vol 29 (11) ◽  
pp. 1445-1451 ◽  
Author(s):  
Ana-Iris Schmatz ◽  
Berthold Streubel ◽  
Elisabeth Kretschmer-Chott ◽  
Andreas Püspök ◽  
Ulrich Jäger ◽  
...  

Purpose Small series with limited follow-up have suggested primary follicular lymphoma of the duodenum (FL-D) to be an indolent disease. We report our experience on a large series of patients followed for a median time period of longer than 6 years. Patients and Methods The study comprised 63 patients with primary FL-D defined as stage I disease. Endoscopy and detailed pathologic work-up was performed at diagnosis and at restaging to monitor the behavior of the neoplastic process. Results Histologically, all 63 patients had FL, low grade (1 to 2). Duodenal endosonography demonstrated lesions confined to mucosa/submucosa in 19 of 20 patients. At an overall median follow-up of 77 months (range, 12 to 177 months), only two untreated patients had developed nodal disease, the remaining 61 patients never experienced extrasmall intestinal disease and large cell transformation did not occur at all. Among 24 patients followed by watch and wait strategy, seven showed spontaneous complete regression and 17 had stable disease; radiotherapy resulted in complete regression in all 19 patients; anti-CD20 antibody monotherapy achieved complete regression in four patients and stable disease in one patient. Various chemotherapy protocols in eight patients caused complete regression in all of them, but local relapses occurred in three. No patients required surgery or died of disease. Conclusion These findings characterize primary FL-D as a remarkably indolent FL variant, which, even left untreated, does not develop tumorous growth, very rarely disseminates (two of 63 patients) and does not transform to high grade disease. A watch and wait approach appears to be the most sensible strategy.


2019 ◽  
Vol 50 (03) ◽  
pp. 188-192
Author(s):  
Saša Ilovar ◽  
Mirjana Benedik ◽  
Tina Vesnaver ◽  
Damjan Osredkar

Background Brain aneurysms are rare in the pediatric population. The diagnosis of a brain aneurysm in a child may be difficult because of its infrequency and often subtle or nonspecific clinical presentation. Endovascular therapy and microsurgical treatment are increasingly used approaches in treating children, possibly contributing to favorable outcomes if patients are treated in a timely manner. Objective We were interested in the clinical presentation, symptoms, diagnostics, treatment, and follow-up of pediatric patients with brain aneurysms in Slovenia. Methods This was a retrospective review of medical documentation of children with intracranial aneurysms treated at the University Children's Hospital in Ljubljana, Slovenia, from January 1998 to December 2017. Results We identified a cohort of eight children (median age: 14.9 years; range: 2.8–17.7). The estimated incidence of pediatric brain aneurysms in Slovenia is 0.12/100,000 children per year. We observed a male predominance (1.7:1). Half of the patients presented with acute onset of neurologic symptoms and three with subarachnoid hemorrhage. One of the patients had a related stroke. The presenting symptoms were tonic–clonic seizures, hemiparesis, paresthesias, speech disturbance, and cranial nerve palsy. The other half of aneurysms were identified incidentally. Five patients had anterior circulation aneurysms; the most prevalent location was the internal carotid artery. One patient was treated with surgical procedures, four patients were treated with endovascular procedures, and three patients were treated conservatively. Outcome was excellent in all patients. Conclusion Endovascular interventions and microsurgical procedures appear to be safe and effective in the treatment of brain aneurysms in the pediatric population. Asymptomatic patients with brain aneurysms need close follow-up.


Author(s):  
Christopher S Graffeo ◽  
Michael J Link ◽  
Scott L Stafford ◽  
Yolanda I Garces ◽  
Robert L Foote ◽  
...  

Abstract BACKGROUND Surgical resection is typically cited as the optimal treatment of patients with Spetzler-Martin Grade I-II arteriovenous malformation (AVM). OBJECTIVE To report our experience with single-fraction stereotactic radiosurgery (SRS) for Spetzler-Martin Grade I-II AVM. METHODS A prospectively maintained registry was reviewed for patients with nonsyndromic Spetzler-Martin Grade I-II AVM having SRS from 1990 to 2011. Patients with <24 mo of follow-up or prior radiotherapy/SRS were excluded, resulting in a study population of 173 patients. Actuarial analysis was performed using the Kaplan-Meier method, and Cox proportional hazards modeling was performed with excellent outcomes (obliteration without new deficits) as the dependent variable. RESULTS Median post-SRS follow-up was 68 mo (range, 24-275). AVM obliteration was achieved in 132 (76%) after initial SRS. Eleven additional patients achieved obliteration after repeat SRS for an overall obliteration rate of 83%. The rate of obliteration was 60% at 4 yr and 78% at 8 yr. Post-SRS hemorrhage occurred in 7 patients (4%), resulting in 3 minor deficits (2%) and 1 death (<1%). Radiation-induced complications occurred in 5 patients (3%), resulting in minor deficits only. One hundred and thirty-seven patients (79%) had excellent outcomes at last follow-up. CONCLUSION SRS is a safe and effective treatment for patients with Spetzler-Martin Grade I-II AVM. Selection bias is likely a contributing factor to explain the superior outcomes generally noted in reported series of microsurgery for patients with low grade AVM.


2020 ◽  
Vol 3 (Supplement_1) ◽  
pp. 155-156
Author(s):  
S Li ◽  
M Monachese ◽  
P James

Abstract Background Endoscopic ultrasound guided fine needle aspiration (EUS-FNA) is the primary method of sampling pancreatic cystic lesions with reported specificity near 100% for diagnosing malignancy. Discrepant positive malignant cytopathology with final surgical pathology of pancreatic cystic lesions has not previously been described. Aims To present a case series and review the literature regarding the implications of positive malignant cytology with discrepant surgical pathology for high risk pancreatic mucinous cystic lesions. Methods Patient demographics, clinical history, procedure details, pathology evaluations and follow-up were collected. A thorough literature review was performed. Results Three patients with high-risk pancreatic cystic lesions on cross-sectional imaging underwent EUS-FNA evaluation. None of these patients had a history of pancreatitis. Cytology was reported as positive for adenocarcinoma in all patients by separate gastrointestinal cytopathologists. All patients underwent surgical resection. The pathology for all resected specimens were reported as intraductal papillary mucinous neoplasm. The resected cysts for two patients demonstrated foci of high-grade dysplasia and the third noted low grade dysplasia. All surgical pathology underwent consensus review by three separate gastrointestinal pathologists. None of the patients were treated with adjuvant chemotherapy. All patients have been followed post-operatively with surveillance magnetic resonance imaging with no evidence of recurrence to date (median follow-up time 239 days, range 133 – 447 days). Conclusions This phenomenon sheds light on the potential for variable interpretations of EUS-FNA cytopathology and surgical resection pathology for high risk pancreatic cystic neoplasms. EUS-FNA may identify foci of adenocarcinoma that is not seen on surgical pathology specimens. Further research is required to examine the long-term outcomes of these patients. Funding Agencies None


2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Arindam Chaudhuri ◽  
Ramita Dey ◽  
Weronika Stupalkowska

Abstract Aim Stent-grafts are finding a place in the treatment of femoral pseudoaneurysms (FpsAs), having been described in the treatment of infected pseudoaneurysms. We present the results of endovascular treatment of non-infected FpsAs using stent-grafts. Methods Case series of patients who underwent stent-graft coverage of FpsAs from January 2016 to December 2020. Stent-graft fractures, occlusions and reinterventions, length of stay (LOS) and 30-day mortality were assessed. Results 11 patients (mean age 75±10.3 years, 7 males; all ASA 3 or 4) underwent stent-graft coverage of FpsAs. Original procedures linked to the formation of FpsA were iliofemoral bypass (n = 3), femorofemoral crossover (n = 3), percutaneous coronary interventions (n = 2), aortobifemoral bypass (n = 1), endovascular aneurysm repair (n = 1) and femoral endarterectomy (n = 1). 3 patients had previous open FpsA repair. Technical success of deployment was 100%. Stent-grafts used included Viabahn Endoprosthesis (WL Gore & Associates, Flagstaff USA; n = 8), Viabahn VBX (WL Gore & Associates; n = 1) and BeGraft (Bentley InnoMed GmbH, Hechingen, Germany; n = 2). All patients were maintained on either antiplatelets or anticoagulant. 10 patients were available for analysis (1 lost to follow-up); radiological follow-up included initial computed tomography angiography and later combined duplex ultrasonography/plain radiography. Median LOS was 4 days (IQR 4). 30-day mortality was 0%. Median survival was 12 months (IQR 17). There was one stent-graft occlusion requiring thrombectomy but no fractures were observed in this series. Conclusions This small series suggests that there may be a role for stent-grafts in treatment of FpsAs in carefully selected patients. Given lack of data on long-term outcomes, continued surveillance is recommended.


2011 ◽  
Vol 8 (6) ◽  
pp. 554-564 ◽  
Author(s):  
Timothy Uschold ◽  
Adib A. Abla ◽  
David Fusco ◽  
Ruth E. Bristol ◽  
Peter Nakaji

Object The heterogeneous clinical manifestations and operative characteristics of pathological entities in the pineal region represent a significant challenge in terms of patient selection and surgical approach. Traditional surgical options have included endoscopic transventricular resection; open supratentorial microsurgical approaches through the midline, choroidal fissure, lateral ventricle, and tentorium; and supracerebellar infratentorial (SCIT) approaches through the posterior fossa. The object of the current study was to review the preoperative characteristics and outcomes for a cohort of patients treated purely via the novel endoscopically controlled SCIT approach. Methods A single-institution series of 9 consecutive patients (4 male and 5 female patients [10 total cases]; mean age 21 years, range 6–37 years) treated via the endoscopically controlled SCIT approach for a pathological entity in the pineal region was retrospectively reviewed. The mean follow-up time was 13.2 months. Results The endoscopically controlled SCIT approach was successfully used to approach a variety of pineal lesions, including pineal cysts (6 patients), epidermoid tumor, WHO Grade II astrocytoma (initial biopsy and recurrence), and malignant mixed germ cell tumor (1 patient each). Gross-total resection and/or adequate cyst fenestration was achieved in 8 cases. Biopsy with conservative debulking was performed for the single case of low-grade astrocytoma and again at the time of recurrence. The mean preoperative tumor and cyst volumes were 9.9 ± 4.4 and 3.7 ± 3.2 cm3, respectively. The mean operating times were 212 ± 71 minutes for tumor cases and 177 ± 72 minutes for cysts. Estimated blood loss was less than 150 ml for all cases. A single case (pineal cyst) was converted to an open microsurgical approach to enhance visualization. There were no operative complications, as well as no documented CSF leaks, additional CSF diversion procedures, or air emboli. Seven patients underwent concomitant third ventriculostomy into the quadrigeminal cistern. At the time of the last follow-up evaluation, all patients had a stable or improved modified Rankin Scale score. Conclusions The endoscopically controlled SCIT approach may be used for the biopsy and resection of appropriately selected solid tumors of the pineal region, in addition to the fenestration and/or resection of pineal cysts. Preoperative considerations include patient presentation, anticipated disease and vascularity, degree of local venous anatomical distortion, and selection of optimal paramedian trajectory.


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