Infrequent Tumor Research Articles

Clinical characteristics and prognosis of pulmonary large cell carcinoma: A population-based retrospective study using SEER data.

BackgroundPulmonary large cell carcinoma (LCC) is an infrequent neoplasm with a poor prognosis. This study explored the clinical characteristics and survival prognostic factors of LCC patients.MethodsPatient data were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. Chi‐square tests or rank‐sum tests were used to compare differences in clinical characteristics. Log‐rank tests, univariate, and multivariate analyses were performed to investigate the independent factors of survival. Analyses of stage I–IV patients were performed to further explore the optimal treatment.ResultsIn total, 3197 LCC patients were included in this analysis. Compared with other non‐small cell lung cancers (NSCLCs), there was a worse overall survival (OS) from LCC. LCC was more common in males, over age 60 and in the upper lobe. A total of 73.6% of patients were stage III/IV. The median OS of stage I–IV patients was 42 months, 22 months, 11 months, and three months, respectively. The elderly, males, later stage, and main bronchus location, or overlapping lesions were risk factors for survival prognosis. In stage I–III patients, treatment including surgery could significantly reduce the risk of death by 60% at least compared with no therapy. Surgery was still beneficial for stage IV patients, and the hazard ratio (HR) compared with no therapy was 0.462 (P = 0.001).ConclusionsOur study concluded that LCC has unique clinical features, and that age, sex, primary site, stage, and treatment are significantly related to OS. Surgery based comprehensive treatments are effective for LCC.Key points Significant findings of the study In stage IV patients, chemotherapy or radiotherapy combined with surgery could further improve survival. When surgical resection involved more than one lobe, it may be beneficial for survival prognosis. What this study adds LCC patients were principally male and over age 60, with later stages and poor survival prognosis. Age, sex, stage, primary site and therapy are closely related to survival.

Update on Renal Neoplasms: Clinicopathologic-Radiologic Correlation With Case-Based Examples.

OBJECTIVE. This article provides a brief overview of the clinicopathologic and radiologic correlation of 12 renal neoplasms, encompassing the conventional subtypes of renal cell carcinoma and a few of the newly recognized subtypes from the 2016 World Health Organization classification of renal tumors. In addition, we touch upon infrequent neoplasms that may enter the differential diagnosis of a renal mass, with corresponding radiologic and gross images and histologic findings of case-based examples. CONCLUSION. Familiarity with the radiologic and pathologic characteristics of renal cell carcinoma and other renal neoplasms is important to correctly identify and treat these masses.

Surgical Treatment on Subungual Osteochondromas in Paediatric Feet: A Case Series Study.

Subungual osteochondroma (SO) is an infrequent and non-malignant bone tumour of the distal phalanx, especially prominent in paediatric populations. The aim of this research was to describe a case series of paediatric feet with SO which received surgical treatments. The secondary purpose was to compare these descriptive data by sex distribution. Methods: Twenty-three paediatric feet with SO confirmed by clinical or radiological features received surgical treatment. Socio-demographic (age, sex, height, weight and BMI) and clinical features (side, location, tumour or pain presence, and nail lift before surgery, as well as recurrence and adverse effects at one month after intervention) were reported. Results: Regarding clinical features before intervention, the most frequent locations of SO were the first toe (86.8%) and the right lower limb (56.5%). In addition, the presence of the tumour, pain and nail lift showed a prevalence of 91.3%, 69.5% and 47% of the study sample, respectively. Considering clinical features at one month after intervention, the most frequent adverse effect was the pain presence (69.5%). In addition, one case (4.4%) presented ulceration. Only one patient (4.4%) suffered from recurrence with a new tumour. There were not statistically significant differences by sex distribution (p > 0.05). Conclusions: This novel study showed that surgery treatment for SO in paediatric populations presented a very low recurrence degree with minor adverse effects and without differences by sex distribution. Thus, further randomized clinical trials should be carried out in order to determine the effectiveness of this intervention in this special population.

Curcumin as an Anticancer Agent in Malignant Mesothelioma: A Review.

Malignant mesothelioma is an infrequent tumor that initiates from the mesothelial cells lining of body cavities. The great majority of mesotheliomas originate in the pleural cavity, while the remaining cases initiate in the peritoneal cavity, in the pericardial cavity or on the tunica vaginalis. Usually, mesotheliomas grow in a diffuse pattern and tend to enclose and compress the organs in the various body cavities. Mesothelioma incidence is increasing worldwide and still today, the prognosis is very poor, with a reported median survival of approximately one year from presentation. Thus, the development of alternative and more effective therapies is currently an urgent requirement. The aim of this review article was to describe recent findings about the anti-cancer activity of curcumin and some of its derivatives on mesotheliomas. The potential clinical implications of these findings are discussed.

Anatomical liver resections for intrahepatic cholangiocarcinomas: Perioperative results and survival

Presenter: Gabriela Ochoa MD | Pontificia Universidad Católica de Chile Background: Intrahepatic cholangiocarcinma (ICC) is an infrequent neoplasm, whose incidence is increasing with a poor prognosis. The only curative management is surgery, however, despite the correct selection of the cases, only 1/3 of patients achieve negative margins. We aimed to describe the experience and results in resected cases from Catholic University Hospital in Chile. Methods: Retrospective cohort analysis of the resected ICC cases with curative intention, between 2006 to 2019. The information was obtained from electronical clinical reports. The analysis was made with descriptive statistics. Results: Out of 17 patients operated for ICC. 10 were males and 7 women, between 48 and 84 years-old, 2 with risk factors (chronic liver disease, iatrogenic bile duct injury). 5 patients received neoadjuvant therapy and in 1 case portal embolization was made beforehand. 13 cases were performed open and 4 laparoscopic. There were 4 extended right hepatectomies, 3 right hepatectomies, 4 extended left hepatectomies, 3 left hepatectomies, 2 bisegmentectomies (IV, V) 1 left lateral sectionectomy. Mean operative time was 263 minutes and hospital stay average was 14,2 days. There was no 30-day mortality. The global morbidity was 41,2% (7 patients), and Clavien Dindo >IIIB complications were 17,6% (3 patients). Reoperations were performed in 2 cases, one for hemoperitoneum and the other for bile leak. The oncologic classification was Ia (1), Ib (1), II (4), IIIa (2), IIIb (8) y IV (1). There were positive lymph nodes in 8 cases and 1 patient with peritoneal metastases. 8 patients resulted R1 (47,1%). 14 patients received adjuvant treatment. The global survival was 82,4% at 6 months, 46,7% at 1 year and 40% at 2 years, with a mean follow-up of 24,3 months. Conclusion: The surgical treatment of ICC normally requires large anatomical liver resections, despite which, the incidence of R1 positive margins is high and could result in the poor prognosis of long-term disease.

An Unusual and Unfortunate Presentation of a Gastric Adenocarcinoma

Gastric adenocarcinoma is the third leading cause of mortality worldwide. Although it is a relatively infrequent neoplasm in North America, it is associated with a high mortality rate. Gastric adenocarcinoma is believed to result from inflammation of gastric cells leading to peptic ulcers which then progresses to cancer. Our case highlights a rare presentation of a rapidly growing gastric adenocarcinoma, originally unseen on endoscopy a month prior, and later appearing as a large circumferential mass. Initially the H. pylori and biopsy results in negative findings for adenocarcinoma, however, a month later the biopsy results were positive for gastric adenocarcinoma. Gastric adenocarcinomas are usually insidious and presents initially as non-specific symptoms such as dyspepsia, nausea, and early satiety. The initial workup consists of an EGD and EUS for localization, tissue biopsy, and determination of tumor size/depth as well as locating regional lymph nodes. Our case highlights the emphasis of frequent surveillance and close follow-up of patients who have increased risk factors for gastric adenocarcinomas as these cancers can often be missed and are fatal.

Orbital hybrid peripheral nerve sheath tumors.

Hybrid peripheral nerve sheath tumors (HPNST) are recently classified tumors from the World Health Organization Classification of soft tissue tumors that display combined features of more than one peripheral nerve sheath tumor. Acknowledgment is important because of its association with the development of neurofibromatosis type 1, type 2, and schwannomatosis. Orbital involvement is rare and only six cases of HPNST have been documented on literature. This article serves to review the pathophysiology, clinical manifestation, diagnosis, treatment, and prognosis of this infrequent but important orbital tumor.

Glandular Odontogenic-The Multifaceted Cyst of the Jaw: An Unusal Case Report

Glandular odontogenic cysts is one of the infrequent neoplasms seen in the oral cavity. They are developmental in origin with unpredictable and aggressive behavior. They are most commonly found in the anterior region of mandible having a predilection for the middle age group. These cysts have striking histologic features even though they have certain overlapping characteristics with other neoplasms. It is very important for us to cognize these unique differentiating features for a conclusive diagnosis. This paper reviews a case of Glandular odontogenic cyst which presented in our department and also elucidates the clinical, radiographical and histological criteria needed to diagnose the same.

Lymphoepithelioma-like carcinoma of the duodenum: a doubly infrequent tumor

Lymphoepithelioma-like carcinoma of the duodenum: a doubly infrequent tumor

Carcinoma ecrino: dos casos de una neoplasia inusual y revisión de la literatura

IntroductionPrimary eccrine carcinomas are rare tumors, representing less than 0.01% of skin cancer, and their behavior is considered aggressive. These types of tumors are clinically nonspecific and, therefore, often misdiagnosed by dermatologists. Then, the histological findings are the key to their correct diagnosis. MethodologyAfter describing two cases of eccrine carcinoma, we performed a review of the literature, using the MEDLINE database, to identify all the cases previously described, analyzing the sex and age of the patients, the description and the location of the tumor and the time of evolution. ResultsWe report a case of eccrine ductal carcinoma with squamous differentiation (EDC) and another case of siringoid eccrine carcinoma (SEC). There are only 22 published cases of ductal eccrine carcinoma with squamous differentiation and 13 cases of CES. The average age of patients with ECD is 72 years old, with a predominance of female sex (61%) and located in head and neck (56.5%). Regarding to SEC, the average age is 51.6 years. 64% are women and 61.5% are located in the head and neck.. ConclusionWe describe two cases of a very infrequent tumor with unusual characteristics and we compared them to previously published cases. Paradoxically, in our cases, the tumors are located in the extremities (armpit and foot) of young patients. According to published literature, PET-CT imaging studies and Mohs surgery would be the reference procedures for the study of extension and treatment of these tumors, respectively.

Meningioma gigante en paciente pediátrico: reporte de caso y revisión de la literatura

Los meningiomas surgen de la cubierta meníngea del sistema nervioso central. Los meningiomas pediátricos son tumoresinfrecuentes que representan del 0,5% al 5% de los tumores del sistema nervioso central en edad pediátrica, siendo el tumor cerebral primario no glial más común. No hay evidencia confirmada de la patogenia de los meningiomas pediátricos. Por lo general, se localizan en la convexidad supra o infratentorial, intraventriculares y en la base del cráneo. La presentación clínica de los meningiomas pediátricos es poco específica y varía según su ubicación y tamaño. Los subtipos de meningiomas en la infancia son principalmente meningoteliales grado I de la OMS (32%). El tratamiento de elección es la resección completa del tumor (Simpson I) para prevenir la recurrencia y mejorar el pronóstico. Presentamos el caso de una niña de 9 años con un gran meningioma frontal, el informe de prevalencia en nuestra institución y una revisión de la literatura actual de esta patología.

LIP OSTEOLIPOMA, CLINICAL AND SURGICAL MANAGEMENT OF AN INFREQUENT DISEASE

Lipomas are relatively infrequent mesenchymal benign neoplasms of oral soft tissues. A wide range of subtypes of lipomas have been previously described, including fibrolipoma, angiolipoma, pleomorphic lipoma, and osteolipoma. The aim of this study is to present a clinical case of an 83-year-old man with a painless labial mass of 20-year duration with a remarkable increase in the size of the lesion in the last 2 years. Oral examination revealed a yellowish and sessile mass located in the lower labial mucosa. The provisional diagnosis was a lipoma, but during its surgical excision, a cartilaginouslike tissue was noticed generating some doubts about our previous diagnosis. The histopathologic findings revealed mature adipocytes surrounding bone tissue and the final diagnosis was osteolipoma. The presence of bony calcifications within this tumor could be related to metaplastic changes during the evolution of the lesion. This rare presentation was previously reported with different oral locations.

Giant pleural fibrous tumor: a case report

Pleural fibrous tumor (PFT) is an infrequent and indolent pleural tumor, and most patients remain asymptomatic for years. The diagnosis is usually incidental and in large-size cases, representing a major surgical challenge. They may be associated to paraneoplastic syndromes such as refractory hypoglycemia or Doege-Potter syndrome. The present case is a 50-year-old female who came with dyspnea, chronic cough and constitutional syndrome. The CT scan showed a large intrathoracic mass of 20×18×17 cm 3 which compressed the right lower and middle lobes (RLL and RML). At the time of the operation, the approach was right lateral thoracotomy extended to the chondrocostal region. During the resection, the tumor was accidentally opened and at that time the patient showed hypoglycemic levels. It is not often a long size PFT with hypoglycemia at the moment of operation without previous diagnosis. PFT is usually asymptomatic and is diagnosed incidentally in half of the cases. Tumor resection is the treatment of choice, which also leads to the cure of paraneoplastic syndrome. For tumors larger than 5 cm, thoracotomy is preferred, and both the tumor and its implant pedicle must be resected with safety margins.

Melanoma anal, patología infrecuente, reporte de un caso

Anal melanoma (MA) is an infrequent tumor, characterized by aggressive behavior and poor prognosis. (1) The nonspecific symptomatology and its similarity with benign anorectal etiologies, makes the diagnosis difficult and leads to a delay in it.(2)(3) Therefore, a thorough physical examination and a high index of suspicion are necessary. The case of a patient who consulted for a non-pigmented and painful anal mass with rectal bleeding is discussed which is diagnosed with anal melanoma.

Giant Cystic Pancreatic Lymphangioma with Mediastinal Extension: A Case Report and Review of the Literature

Lymphangioma is an infrequent benign tumour that is formed usually from a congenital malformation of the lymphatic ducts causing lymphangiectasis. The abdominal location represents 1-5%, with dominance in the mesentery and retroperitoneum, however, the pancreatic location is very rare having described less than 100 cases published worldwide. The clinical symptoms of the lymphangiomas are non-specific and depend of the tumour’s size and location. Here we present a clinical case of a pancreatic lymphangioma with mediastinal extension who was treated with a complete resection and this case shows that the diagnosis of cystic pancreatic lymphangioma must be taken as a differential diagnosis of pancreatic cystic lesions.

Sertoli-Leydig Cell Tumor (SLCT) of the Ovary - A Case Report

Sertoli-Leydig Cell Tumor (SLCT) is an extremely rare and distinctive sex cord-stromal tumor. Ovarian sex cordstromal tumors are infrequent tumors that differ from the more frequent epithelial neoplasm and have strong association with hormone-mediated syndromes, presentation in a wide age range, and the diagnosis is usually at early-stage disease with a good outcome. SLCT typically present with pelvic or abdominal pain and tenderness, mass, and or abnormal menstruation, and with hormonal activity reported predominantly after menarche. We report a case of 18-year-old unmarried female presented with severe pain and lump in the lower abdomen and also a case of primary amenorrhea with normal secondary sex characters. She was a suspected case of twisted ovarian tumor on USG and underwent laparotomy followed by total excision of the lump and histopathology report revealed Sertoli- Leydig Cell Tumor. Though it is a rare tumor, the possibility of Sertoli-Leydig Cell Tumor in a young woman should be kept in mind. Faridpur Med. Coll. J. Jul 2018;13(2): 104-107

Targeting Angiogenesis in Pancreatic Neuroendocrine Tumors: Resistance Mechanisms.

Despite being infrequent tumors, the incidence and prevalence of pancreatic neuroendocrine tumors (P-NETs) has been rising over the past few decades. In recent years, rigorous phase III clinical trials have been conducted, allowing the approval of several drugs that have become the standard of care in these patients. Although various treatments are used in clinical practice, including somatostatin analogues (SSAs), biological therapies like sunitinib or everolimus, peptide receptor radionuclide therapy (PRRT) or even chemotherapy, a consensus regarding the optimal sequence of treatment has not yet been reached. Notwithstanding, sunitinib is largely used in these patients after the promising results shown in SUN111 phase III clinical trial. However, both prompt progression as well as tumor recurrence after initial response have been reported, suggesting the existence of primary and acquired resistances to this antiangiogenic drug. In this review, we aim to summarize the most relevant mechanisms of angiogenesis resistance that are key contributors of tumor progression and dissemination. Furthermore, several targeted molecules acting selectively against these pathways have shown promising results in preclinical models, and preliminary results from ongoing clinical trials are awaited.

412P - Updated analysis of the national registry of nervous system tumours in Spain (RETSINE). RETSINE: National registry of nervous system tumours of the Spanish research group in neuro-oncology (GEINO) and Spanish infrequent and orphan tumours group (GETHI)

BackgroundThe Spanish Research Group in Neuro-oncology (GEINO) and Spanish Infrequent and Orphan Tumors Group (GETHI) initiated a Nervous System Tumors Registry (RETSINE) in September 2017 with the aim of evaluating accurate, basic data concerning this oncological pathology in Spain. (Spanish Agency of Medicines and Medical Devices code: GG-TIN-2017-01). MethodsThe RETSINE database collects demographic, diagnostic and treatment data from all the hospitals participating in the project. Secondarily, each researcher indicates if there is a tumor sample available for future studies that arise from the registry. ResultsFrom September 2017 to April 2019, the registry has included 1021 patients (p) from 26 different hospitals. P have been included at a rate of 60 per month. With a median age of 60 y (range: 16-89), there is a discrete predominance in males (56.1%). The main symptoms at presentation are seizures, cognitive impairment and ataxia (26.1%, 16.8% and 6.6% of p). Histology provided in 841 p=764/841 glioma (90,8%) [High Grade Glioma 618/764 (80.9%), Low Grade Glioma 146/764 (19.1%)], 77/308 no glioma (9,2%). Primary surgical treatment= 42.4% total tumor removal; 38.9% subtotal tumor removal and 7.7% biopsy. The median overall survival is 18.1 m (95% CI 15.5-20.6): 115.6 m (95% CI 38.8-192.4) in Low Grade and 17.5 m (95% CI 15.4-19.5) in High Grade Glioma. ConclusionsRETSINE is a valuable tool for analyzing the current situation of Nervous System Tumors in Spain. The possibility of carrying out clinical studies in tumors of low incidence will be increased and improved with records of cases with associated biological sample. The database provides medical information that can also assist the orientation of healthcare policies. Legal entity responsible for the studySpanish Research Group in Neuro-oncology (GEINO) and Spanish Infrequent and Orphan Tumors Group (GETHI). FundingHas not received any funding. DisclosureAll authors have declared no conflicts of interest.

Xantogranuloma de la Región Selar: Reporte de 3 casos y revisión de la literatura

El xantogranuloma corresponde a un tumor infrecuente que comparte diversas características con otros tumores de la región selar. Dada la escasa cantidad de casos reportados, la experiencia en el reconocimiento y abordaje de este tumor es limitada. Se presenta el marco teórico de la patología, buscando destacar sus características principales, señalar los hallazgos clínicos e imagenológicos que permiten sospechar el diagnóstico, describir el pronóstico tras la cirugía, y establecer sus diferencias frente a otros tumores de la región selar. Además se describe la experiencia quirúrgica desarrollada por los autores en tres casos de xantogranuloma de la región selar tratados en el Hospital Regional de Concepción entre los años 2013 y 2015.

Huge mediastinal teratoma in children: anesthesiological approach, surgical strategy and literature review

Teratomas of the anterior mediastinum are infrequent germ cell tumour. These tumours occur at all ages, but are rarely discovered before puberty. We report a case of anterior mediastinal mature teratoma in a baby girl aged 5 months who presented to us with recurrent infectious pneumonia associated with hemoptysis since one month. Computed tomographic scan of thorax revealed a large anterior mediastinal mass measuring 8.01×6.72×7.28 cm 3 with calcification within. The authors report this current case to emphasize the importance of the multidisciplinary care and perioperative approach between anaesthesiologist and surgeon to a successful management of a huge mediastinal mature teratoma in an infant. Tips, tricks and pitfalls are proposed in this paper for a surgical outcome effective and no clinical complication.