scholarly journals Chordoma: A Review and Differential Diagnosis

Author(s):  
Veronica Ulici ◽  
Jesse Hart

Context.— Chordomas are uncommon malignant neoplasms with notochordal differentiation encountered by neuropathologists, bone/soft tissue pathologists, and general surgical pathologists. These lesions most commonly arise in the axial skeleton. Optimal therapy typically involves complete surgical resection, which is often technically difficult owing to the anatomic location, leading to a high rate of recurrence. Lesions have been generally resistant to radiation and chemotherapy; however, experimental studies involving targeted therapy and immunotherapy are currently underway. Objective.— To summarize the clinical and pathologic findings of the various types of chordoma (conventional chordoma, dedifferentiated chordoma, and poorly differentiated chordoma), the differential diagnosis, and recent advances in molecular pathogenesis and therapeutic modalities that are reliant on accurate diagnosis. Data Sources.— Literature review based on PubMed searches containing the term “chordoma” that address novel targeted and immunomodulatory therapeutic modalities; ongoing clinical trials involved in treating chordoma with novel therapeutic modalities identified through the Chordoma Foundation and ClinicalTrials.gov; and the authors' practice experience combined with various authoritative texts concerning the subject. Conclusions.— Chordoma is a clinically and histologically unique malignant neoplasm, and numerous diagnostic considerations must be excluded to establish the correct diagnosis. Treatment options have largely been centered on surgical excision with marginal results; however, novel therapeutic options including targeted therapy and immunotherapy are promising means to improve prognosis.

Author(s):  
S. K. Yarovoy ◽  
I. B. Shikina

Aim: to conduct a pharmacoeconomic analysis of chemotherapy taking into account the types of malignant neoplasms of the bronchi and lung, and to calculate pharmacoeconomic efficiency.Materials and methods. The materials for the study were the data of the Moscow Cancer Hospital n.a. D.D. Pletnev for 2019. The cost of drugs was calculated according to the data of the website aptekamos.ru (for June 2019).Results and discussion. For 300 patients with squamous non-small cell lung cancer, it is required 3205.5 courses of chemotherapy of I (1747.5) and II (1458) lines. For 200 patients with non-small cell non-squamous EGFR positive lung cancer, it is required 1,413.1 courses of chemotherapy and targeted therapy. For 70 patients with non-squamous cell non-small cell lung cancer with ALK mutation, it is required 450.2 courses of chemotherapy and targeted therapy. For 280 patients with non-squamous cell non-small cell lung cancer lung cancer without mutations, it is required 7515 chemotherapy courses. For 150 patients with small cell lung cancer, it is required 1,656.8 courses of chemotherapy. Thus, a total sample of 1000 patients, excluding the type of malignant neoplasm of broncus and lung are required 14,239.8 chemotherapy courses. The determined cost of 67,000 rubles for the average chemotherapy course per patient takes into account the necessary colony stimulating factors, antiemetic, infusion solutions, etc.; in the case of treatment with erlotinib (the length of the course is 8 months), the cost is 663,408.0 rubles; in the case of treatment with crizotinib (the length of the course is 11 months course), the cost is 2044110.0 rubles. The average cost of drug therapy for one patient with malignant neoplasm of bronchus and lung is 1136155.9 rubles.Conclusion. We determined the pharmacoeconomic dependence of a chemotherapy on the type of malignant neoplasms of bronchus and lung and the main anticancer drug used. The median of the overall survival rate of patients with malignant neoplasms of bronchus and lung was 42 months. The average cost of drug therapy was 27051.3 rubles per month. The median survival rate without therapy is 3 months. Thus, therapy can extend the patient’s life by an average of 39 months. The cost of a month of a saved life is 29132.2 rubles. 


Sarcoma ◽  
2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
LM. Chinchilla-Tábora ◽  
J. Ortiz Rodríguez-Parets ◽  
I. González Morais ◽  
J. M. Sayagués ◽  
M. D. Ludeña de la Cruz

Ewing sarcomas are an uncommon group of malignant neoplasms. A multidisciplinary approach is highly recommended to reach a correct diagnosis, considering the clinical, radiological, and histopathological aspects. Since in up to 90% of cases, the translocation t (11; 22) (q24; q12) occurs resulting in a chimeric fusion transcript EWSR1-FLI-1. The pathologist has several tools in addition to conventional techniques (hematoxylin and eosin), such as immunohistochemistry, which plays a very important role in the differential diagnosis. We present a series of 15 cases of molecularly confirmed ES, in which we found a sensitivity of 100% for CD99 and 80% for PAX8 by immunohistochemistry. This indicates a high sensitivity; however, it is known that both CD99 and PAX8 are also expressed in other tumours. Therefore, molecular confirmation should be performed in all cases.


2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
Takahiko Nakajima ◽  
Haruo Yagi ◽  
Hayato Baba ◽  
Takashi Minamisaka ◽  
Shigeharu Miwa ◽  
...  

Pseudomalignant erosion is a diagnostic pitfall for pathologists in the differential diagnosis of malignant neoplasms. Here, we present a challenging case of a biopsy specimen from the eroded head of a polyp at the esophagogastric junction. A malignant neoplasm could not be ruled out due to the presence of bizarre stromal cells. A second biopsy performed after the administration of a proton pump inhibitor (PPI) for 4 weeks revealed endoscopic resolution of the polyp along with the complete histological resolution of the bizarre stromal cells and led to the diagnosis of pseudomalignant erosion in a reflux gastroesophageal polyp. In conclusion, histological and endoscopic response to PPI therapy is an important clue for the correct diagnosis of reflux gastroesophageal polyps with pseudomalignant erosion.


2016 ◽  
Vol 12 (1) ◽  
pp. 13-24 ◽  
Author(s):  
Katie Ekberg ◽  
Markus Reuber

There are many areas in medicine in which the diagnosis poses significant difficulties and depends essentially on the clinician’s ability to take and interpret the patient’s history. The differential diagnosis of transient loss of consciousness (TLOC) is one such example, in particular the distinction between epilepsy and ‘psychogenic’ non-epileptic seizures (NES) is often difficult. A correct diagnosis is crucial because it determines the choice of treatment. Diagnosis is typically reliant on patients’ (and witnesses’) descriptions; however, conventional methods of history-taking focusing on the factual content of these descriptions are associated with relatively high rates of diagnostic errors. The use of linguistic methods (particularly conversation analysis) in research settings has demonstrated that these approaches can provide hints likely to be useful in the differentiation of epileptic and non-epileptic seizures. This paper explores to what extent (and under which conditions) the findings of these previous studies could be transposed from a research into a routine clinical setting.


2019 ◽  
pp. 57-67
Author(s):  
A. N. Katrich ◽  
V. A. Porkhanov ◽  
N. S. Ryabin

Objective: efficacy evaluation of the CEUS LI RADS v2017® system for differential diagnosis of liver tumors in patients with and without cirrhosis.Materials and methods. Retrospective analysis of diagnostic results of the 165 patients with liver tumors (177 nodules) was done. All patients underwent CEUS with results interpretation in accordance to the CEUS LIRADSv2017 ® criteria. Patients were divided into 2 groups based on clinical and morphological data. Group 1 included 62 patients with cirrhosis and/or CVH. Group 2 included 110 patients without risk factors for HCC.Results. Diagnostic efficiency of CEUS LI RADS v2017® for HCC identification was: group 1 – Se – 100%, Sp – 88%, Ac – 95.5%; group 2 – Se – 100%, Sp – 68.8%, Ac – 72.7%; general group Se – 100%, Sp – 72.2%, Ac – 81.4%. In the 2nd group, 21 out of 22 neoplasms, confirmed morphologically as FNH, we classified as LR 4. By applying benign character and specific contrasting patterns of FNG, they were transferred from LR 4 to LR 3. This allowed to increase sensitivity and specificity of differential diagnosis in group 2 (Se – 100%, Sp – 90.6%, Ac – 91.8%) and in general group (Se – 100%, Sp – 90.1%, Ac – 93.2%). Diagnostic efficiency of the criteria for non hepatocellular malignant neoplasms (LR M) was: group 1 – Se – 77.8%, Sp – 100%, Ac – 97%; group 2 – Se – 90%, Sp – 96.7%, Ac – 93.6%; general group- Se – 88.1%, Sp – 98.3%, Ac – 94.9%.Conclusion. Our study confirmed high accuracy of the CEUS LI RADS v2017® system in the differential diagnosis of focal liver tumors. Modification of the system (in particular, transfer of typical FNG forms from the LR 4 category) will make it possible to increase the accuracy of diagnostics by 20%. It will allow to use the LI RADS v2017® system for interpretation CEUS not only among patients with liver cirrhosis, but also in a general group without risk factors of GCC.


Molecules ◽  
2021 ◽  
Vol 26 (11) ◽  
pp. 3143
Author(s):  
Sergey E. Parfenyev ◽  
Sergey V. Shabelnikov ◽  
Danila Y. Pozdnyakov ◽  
Olga O. Gnedina ◽  
Leonid S. Adonin ◽  
...  

Breast cancer is the most frequently diagnosed malignant neoplasm and the second leading cause of cancer death among women. Epithelial-to-mesenchymal Transition (EMT) plays a critical role in the organism development, providing cell migration and tissue formation. However, its erroneous activation in malignancies can serve as the basis for the dissemination of cancer cells and metastasis. The Zeb1 transcription factor, which regulates the EMT activation, has been shown to play an essential role in malignant transformation. This factor is involved in many signaling pathways that influence a wide range of cellular functions via interacting with many proteins that affect its transcriptional functions. Importantly, the interactome of Zeb1 depends on the cellular context. Here, using the inducible expression of Zeb1 in epithelial breast cancer cells, we identified a substantial list of novel potential Zeb1 interaction partners, including proteins involved in the formation of malignant neoplasms, such as ATP-dependent RNA helicase DDX17and a component of the NURD repressor complex, CTBP2. We confirmed the presence of the selected interactors by immunoblotting with specific antibodies. Further, we demonstrated that co-expression of Zeb1 and CTBP2 in breast cancer patients correlated with the poor survival prognosis, thus signifying the functionality of the Zeb1–CTBP2 interaction.


2013 ◽  
Vol 57 (7) ◽  
pp. 566-570 ◽  
Author(s):  
Mariana F. Guzzo ◽  
Cristina B. Formiga Bueno ◽  
Thiago T. Amancio ◽  
Sergio Rosemberg ◽  
Cleonice Bueno ◽  
...  

Intracranial germinomas (GE) are malignant neoplasms most commonly found in the suprasellar region, which may cause anterior and particularly posterior pituitary hormone deficits with central diabetes insipidus (DI). Differential diagnosis of pituitary stalk thickening includes granulomatous, inflammatory, infectious, and neoplastic lesions. Although careful analysis of clinical, laboratory, and imaging findings may facilitate the diagnosis, transsphenoidal biopsy is indicated to confirm the disease, as the correct diagnosis directs the appropriate treatment.


2021 ◽  
Author(s):  
Tommaso Panici Tonucci ◽  
Andrea Sironi ◽  
Eleonora Pisa ◽  
Benedetta Di Venosa ◽  
Luigi Bonavina

Summary Background Schwannoma is a benign tumor arising from Schwann cells of the peripheral nerves. It is often asymptomatic and can develop in the retroperitoneum, mediastinum, head and neck region, and upper and lower extremities. Schwannoma of the abdominal wall is extremely rare, but differential diagnosis with malignant neoplasms is important to reduce the risk of undertreatment. Methods A narrative review of abdominal wall schwannoma was performed using PubMed, EMBASE, and Web of Science database and the search terms “schwannoma”, “neurinoma”, “neurilemmoma”, “soft tissue tumors”, “neurogenic tumor”, “rectus abdominis mass”, “abdominal wall”. In addition, the hospital charts were reviewed to report the personal experience. Results Only 9 single case-reports of benign schwannoma of the abdominal wall were found in the English medical literature over the past decade. None of the patients received preoperative biopsy and all were resected with clear margins. In addition to the literature review, we report the case of a 58-year-old man referred for a palpable mass in the left upper abdominal quadrant. Ultrasonography and magnetic resonance imaging revealed a solid and well-encapsulated mass inside the left rectus abdominis muscle. A core biopsy of the lesion provided the diagnosis of cellular schwannoma and this was confirmed by histopathologic examination of the surgical specimen. Conclusions Benign schwannoma of the abdominal wall is extremely rare. Percutaneous core needle biopsy is important for the differential diagnosis with more common and biologically more aggressive malignancies, such as desmoid tumors and sarcomas, and may be relevant for planning the most appropriate management.


2012 ◽  
Vol 2012 ◽  
pp. 1-11 ◽  
Author(s):  
Yang Zhang ◽  
Karen Goddard ◽  
John J. Spinelli ◽  
Carolyn Gotay ◽  
Mary L. McBride

We conducted a population-based retrospective study to assess the long-term risks of overall and cause-specific mortality and second malignant neoplasm (SMN) among survivors of young adult cancer compared to the risk in British Columbia (BC) population and to evaluate the effects of demographic and clinical factors on risk. 1248 5-year survivors of young adult cancer diagnosed 1970–1995 between 20 and 24 years of age were identified from the BC Cancer Registry and followed to the end of 2007. Standardized mortality ratios (SMRs) and standardized incidence ratios (SIRs) were calculated. The Cox proportional hazards model was used to estimate the effects of different demographic and disease-related characteristics on the risk of death and SMN. A total of 138 deaths and 62 SMNs were observed during follow-up. The overall SMR was 5.9 (95% CI 4.9–6.9) and the absolute excess risk was 5.3 per 1,000 person-years. The overall SIR was 3.0 (95% CI 2.3–3.8). Treatment with radiation resulted in increased risks of death and SMN. These observed increased risks emphasize the importance of prevention, surveillance, and treatment of late effects in survivors of young adult cancers.


PEDIATRICS ◽  
1996 ◽  
Vol 98 (3) ◽  
pp. 461-463
Author(s):  
Gary L. Darmstadt

Genital warts are common relative to other verrucous lesions of the anogenital region. Consideration of the differential diagnosis of verrucous anogenital lesions is necessary, however, to make a correct diagnosis consistently and to avoid futile, painful, and possibly traumatic attempts at treatment. In this report, I describe a child with a history suspicious for sexual abuse who was treated with inappropriate measures because of misdiagnosis of perianal lymphangioma circumscriptum as genital warts. The pathogenesis, differential diagnosis, and treatment of lymphangioma is presented. CASE REPORT A 5-year-old Hispanic boy was referred by his pediatrician to the Dermatology Service with an eruption of red papules on the left buttock and perianal region.


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