scholarly journals Extra-axial dedifferentiated chordoma: A case report with brachyury immunohistochemical confirmation, literature review and pathologists’ perspective

2016 ◽  
Vol 2 (3) ◽  
pp. 161
Author(s):  
Evita Bonita Henderson-Jackson ◽  
Jaime Caracciolo ◽  
Douglas Letson ◽  
Marilyn Bui
Keyword(s):  
Radiation Therapy ◽  
Case Report ◽  
Relevant Literature ◽  
Exceptional Case ◽  
High Grade ◽  
Malignant Bone Tumor ◽  
Diagnostic Challenge ◽  
Primary Tumors ◽  
Axial Location ◽  
First Case

<p>Dedifferentiated chordoma is a rare and aggressive malignant bone tumor. It is known as a variant of conventional chordoma, which possesses additional high-grade sarcomatous elements. Dedifferentiated chordoma is commonly identified in axial location as recurrences, or following radiation therapy of primary tumors. Prognosis is poor and there is a potential risk for metastasis. There have been only a few reports of primary dedifferentiated chordoma. Owing to its rarity, especially when it is in an unusual extra-axial location, primary dedifferentiated chordoma presents a diagnostic challenge. Brachyury, a recently described immunohistochemical marker specific for chordoma, has improved the diagnostic accuracy of chordoma and its variants in extra-axial sites. We herein report an exceptional case of extra-axial dedifferentiated chordoma confirmed by the expression of brachyury, the first case report of this kind. The review of relevant literature and discussion of practical diagnostic approach from sarcoma pathologists’ perspective are intended to provide an update of this topic. </p>

The first case of BRG (SMARCA4)/INI Deficient Tracheal Carcinoma: A Case Report and Review of the Literature

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S150-S151
Author(s):  
A C Omo-Ogboi ◽  
W Wang ◽  
B Zhao
Keyword(s):  
Case Report ◽  
High Grade ◽  
Primary Tumors ◽  
First Case ◽  
Tobacco Abuse ◽  
History Of ◽  
Classification Of Tumors ◽  
Poorly Differentiated

Abstract Introduction/Objective Primary tumors of the trachea are rare, they account for less than 0.1% of tumors in humans. In adults, 90% of primary tracheal tumors are malignant, with squamous cell carcinoma and adenoid cystic carcinoma accounting for two-thirds, with other forms occurring less frequently. The BRG (SMARCA4)/INI deficient tumor is a relatively new defined entity which is recently introduced in the WHO Classification of Tumors, 5th edition, 2021. The gene SMARCA4 is located at 19p13. Loss of SMARCA4 has been reported in several aggressive tumors with high- grade undifferentiated rhabdoid morphology but has not been reported in the trachea. Hence, we report the first case of BRG (SMARCA4)/ INI deficient tracheal carcinoma. Methods/Case Report We present a 60-year-old male with a history of tobacco abuse, shortness of breath, and a tracheal mass on chest imaging. Bronchoscopy was performed and showed a fleshy friable lesion at the anterior trachea with evidence of blood dripping into the distal airways. Results (if a Case Study enter NA) Microscopic examination showed a high grade, poorly differentiated carcinoma with tumor necrosis, high mitotic counts, and marked nuclear pleomorphism. Immunohistochemical stains were performed. The tumor cells were strongly and diffusely positive for CK-7 and weakly positive for synaptophysin, negative for pan-cytokeratin, TTF-1, CK-20, p40, CK5/6, and chromogranin. Then BRG (SMARCA4) and INI 1 (BAR47) were performed and showed negative staining on BRG expression, while INI 1 is intact (nuclear expression). These features are consistent with BRG (SMARCA4)/ INI deficient carcinoma. Conclusion BRG (SMARCA4)/ INI deficient carcinoma is a new entity in the trachea, which is very aggressive with a poor prognosis. Targeted therapy or clinical trials may be available as additional cases are diagnosed in the future.

Successful Laser-assisted Excision of a Metastatic Midbrain Tumor

Neurosurgery ◽  
1986 ◽  
Vol 18 (6) ◽  
pp. 795-797 ◽  
Author(s):  
William D. Tobler ◽  
Raymond Sawaya ◽  
John M. Tew
Keyword(s):  
Radiation Therapy ◽  
Case Report ◽  
Clinical Course ◽  
Metastatic Adenocarcinoma ◽  
Computed Tomographic ◽  
Metastatic Lesions ◽  
First Case ◽  
Autopsy Series ◽  
Solitary Brain Metastases

Abstract Metastatic lesions to the midbrain are rare. They are found in 1 to 3% of autopsy series of solitary brain metastases. The consensus of opinion in the current literature is that they are inoperable lesions and should be treated by radiation therapy alone. This is the first case report of a completely excised metastatic adenocarcinoma to the midbrain. The patient's clinical course has been stable, and there is no computed tomographic evidence of recurrence at 18 months follow-up. (18:795-797, 1986)

scholarly journals Glottic Web Formation During Radiation Therapy: First Case Report Compared With Radiation Dose

2019 ◽  
Vol 98 (9) ◽  
pp. 543-544
Author(s):  
Naoya Ishibashi ◽  
Toshiya Maebayashi ◽  
Takuya Aizawa ◽  
Masakuni Sakaguchi ◽  
Atsuo Ikeda
Keyword(s):  
Radiation Therapy ◽  
Case Report ◽  
Radiation Dose ◽  
First Case

First case of intracaval recurrence of high‐grade endometrial stromal sarcoma: A case report

2021 ◽  
Author(s):  
Vincenzo Dario Mandato ◽  
Riccardo Valli ◽  
Monica Silvotti ◽  
Valentina Mastrofilippo ◽  
Giovanni Casali ◽  
...  
Keyword(s):  
Case Report ◽  
Endometrial Stromal Sarcoma ◽  
High Grade ◽  
Stromal Sarcoma ◽  
First Case

scholarly journals Synchronous Primary Tumors of Kidney and Bilateral Ovaries-A Diagnostic Challenge: Case Report

2020 ◽  
Vol 2 (3) ◽  
Author(s):  
Kriti Singh ◽  
Deepti Gupta ◽  
Anjali Tewari ◽  
Vinod Kumar Mudgal ◽  
Nupur Trivedi ◽  
...  
Keyword(s):  
Gastrointestinal Tract ◽  
Clinical Characteristics ◽  
Case Reports ◽  
Indian Literature ◽  
Diagnostic Challenge ◽  
Primary Tumors ◽  
Multiple Primaries ◽  
First Case ◽  
Primary Neoplasm ◽  
Gynaecological Malignancies

Multiple malignancies in the same patient account for 2% to 17% of all cancers. However, multiple synchronous primary tumours in the same patient are extremely rare. Most synchronous primary tumors involve the genitourinary and gastrointestinal tract, followed by both breast and genitourinary tract, and breast and gastrointestinal tract. Among gynaecological malignancies, synchronous primary carcinomas of the endometrium and ovary are the commonest. Synchronous primary neoplasm involving kidney and ovaries are extremely rare, with only few case reports in the literature; however, there are no reported cases in the Indian literature. We hereby report a case of 60 year old woman who underwent radical nephrectomy for renal cancer and primary cytoreductive surgery for her ovarian cancer. In this report we present the first case in the Indian literature of synchronous primary cancers in right kidney and bilateral ovaries. Based on the combination of factors (diagnosis, treatment and demographics), it is expected that in the course of the coming years, the prevalence of patients with multiple primaries will increase. Careful attention should be paid to the differential diagnosis between double primary and metastatic tumours, based on the pathologic, radiological and clinical characteristics.

scholarly journals Case Report: Five Adult Cases of H3K27-Altered Diffuse Midline Glioma in the Spinal Cord

2021 ◽  
Vol 11 ◽  
Author(s):  
Quanquan Gu ◽  
Yajing Huang ◽  
Hao Zhang ◽  
Biao Jiang
Keyword(s):  
Spinal Cord ◽  
Case Report ◽  
Aggressive Behavior ◽  
Progression Free Survival ◽  
Central Canal ◽  
Rapid Progression ◽  
High Grade ◽  
First Case ◽  
Diffuse Midline Glioma

BackgroundDiffuse midline glioma with H3K27-altered (DMG-H3K27a) is a novel tumor entity of the pediatric-type diffuse high-grade tumor in the latest WHO CNS 5. It mostly affects children and is only rarely found in adults. The tumor has a high level of aggressiveness, with a rapid progression and bad prognosis. In adults, the spinal cord is the most common site of DMG-H3K27a. Rare adult cases of primary DMG-H3K27a in the spinal cord were reported in this study, together with clinico-histopathologico-radiographic data.MethodsFrom January 2016 to December 2020, we conducted a retrospective study of five adults with primary DMG-H3K27a in the spinal cord, analyzing their clinical, pathohistological, and radiographic datasets from the first diagnosis to follow-up.ResultsAll five patients were diagnosed for the first time and were given full treatment. In three of the five patients, post-operative follow-up revealed tumor recurrence. The longest survival of the five patients was 45 months at the time of report submission, while the longest progression-free survival (PFS) following surgery was 20 months. Immunohistochemical studies showed the tumors featured aggressive behavior (grade 4) and were positive for the H3K27M mutation. The radiographic appearances were varied, but they were all initially mistaken as benign. DMG-H3K27a in the spinal cord was characterized by isointense/hyperintense on T1WI and isointense/hyperintense on T2WI, as well as cystic necrosis and peripheral spinal cord edema, as well as central canal enlargement and other types of enhancement.ConclusionThis is the first case report focusing on adult DMG-H3K27a of the pediatric-type diffuses high-grade gliomas in the spinal cord. In our cases, we discovered the following: 1) adults had a better prognosis with a longer PFS compared with prior pediatric reports; 2) despite aggressive behavior under the microscope, radiographic appearances of the tumors were less aggressive; and 3) adjuvant treatment, including TCM, may have played a role in the prognosis.

scholarly journals A Giant Egg-like symptomatic Loose Body in the Peritoneal Cavity: A Case Report

1970 ◽  
Vol 29 (6) ◽  
Author(s):  
Berhanetsehay Teklewold ◽  
Anteneh Kehaliw ◽  
Melat Teka ◽  
Bereket Berhane
Keyword(s):  
Abdominal Pain ◽  
Case Report ◽  
Incidental Finding ◽  
Loose Body ◽  
Loose Bodies ◽  
Diagnostic Challenge ◽  
First Case ◽  
Appendices Epiploicae ◽  
The One ◽  
High Index Of Suspicion

Background: Peritoneal loose bodies are rare lesions that are usually found as an incidental finding during abdominal surgery. Large loose bodies, measuring more than 5 cm, are rare and only a few cases are reported in the literature. Peritoneal loose bodies are usually infarcted appendices epiploicae, which become detached and appear as a peritoneal loose body in the abdominal cavity.Case Presentation: We report here the first case, in the local Ethiopian context, of a giant “egg-like” loose peritoneal body measuring 7 × 6 cm found in a 50-year-old man who presented with a cramping abdominal pain and features of abdominal obstruction. The current hypothesis as regards these bodies and the diagnostic challenges is discussed.Conclusion: Small peritoneal loose bodies are common but giant and symptomatic ones’, like the one discussed here, are very rare and a diagnostic challenge. And, in the context of intestinal obstruction, a high index of suspicion is needed in order to diagnose them.

scholarly journals Malignant melanoma oral cavity: case report of two patients from sub-Himalayan region

2021 ◽  
Vol 7 (5) ◽  
pp. 896
Author(s):  
Jyoti Sharma ◽  
Manish Gupta ◽  
Amit Saini
Keyword(s):  
Radiation Therapy ◽  
Case Report ◽  
Malignant Melanoma ◽  
Oral Cavity ◽  
Male Patient ◽  
Radiation Treatment ◽  
Post Surgery ◽  
First Case ◽  
Pigmented Lesion ◽  
Patient Reported

<p class="abstract">Malignant melanoma of the oral cavity is an exceedingly rare tumor representing 0.2 to 8% of all melanomas. Mucosal melanomas are extremely rare and aggressive neoplasms. Patient reporting to the clinician with a pigmented lesion should raise suspicion in the first visit itself and should be further investigated so as to detect this dreaded malignancy at an earlier stage and thus managed appropriately. We presented two such rare cases who reported at our centre treated with different modalities and had different responses to treatment. In first case report 65 year old male patient diagnosed with malignant melanoma of left upper alveolus underwent multiple modalities of treatment like surgery, chemotherapy, radiation therapy but unfortunately as he defaulted post-surgery and also due to COVID-19 lockdown restrictions he was treated in various centres and finally the result was inoperable residual gigantic mass resistant to chemotherapy and radiation therapy. In second case report, 82 year old male patient reported with malignant melanoma hard palate having good response to initial chemotherapy. He was planned on hypo-fractionated radiotherapy in view of his old age but he refused radiation treatment and is on oral temozolamide and thalidomide with stable disease and good quality of life since past 6 months.</p>

scholarly journals Epithelioid Variant of High-Grade Myxofibrosarcoma in the False Vocal Fold: A Case Report

2021 ◽  
Vol 64 (12) ◽  
pp. 954-958
Author(s):  
Seulki Song ◽  
Dae Hyun Song ◽  
Jin Pyeong Kim
Keyword(s):  
Case Report ◽  
Vocal Fold ◽  
Surgical Margin ◽  
High Rate ◽  
High Grade ◽  
Large Tumor ◽  
Histologic Subtype ◽  
First Case ◽  
False Vocal ◽  
Epithelioid Variant

Myxofibrosarcoma (MFS) is a histologic subtype of malignant fibrous histiocytoma (MFH), with a predominant myxoid component. MFS is characterized by locally aggressive behavior and a high rate of local recurrence, however, with a good prognosis. Head and neck MFS accounts for 3% of all cases of MFS. To date, only two cases of laryngeal MFS have been reported. Owing to the rarity of MFS, the clinical characteristics and optimal treatment options remain controversial. Surgical resection with a clear margin is considered the treatment of choice. Compared to traditional MFS tumors, epithelioid variants have worse prognosis. Other factors associated with a poor prognosis of MFS tumors include inadequate surgical margins, large tumor size, old age, and high-grade tumors. Herein, we report a case of high-grade epithelioid variant MFS located in the false vocal fold, requiring total laryngectomy to obtain an adequate surgical margin. To our knowledge, this is the first case report of epithelioid variant of high-grade MFS presenting in the larynx.

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