scholarly journals CS-6 A case of poorly differentiated chordoma with systemic metastasis

2021 ◽  
Vol 3 (Supplement_6) ◽  
pp. vi28-vi28
Author(s):  
Naoki Shinojima ◽  
Keisuke Harada ◽  
Yuji Dekita ◽  
Haruaki Yamamoto ◽  
Mai Itouyama ◽  
...  
Keyword(s):  
Tumor Resection ◽  
Residual Tumor ◽  
Final Diagnosis ◽  
High Dose ◽  
Local Doctor ◽  
Short Period ◽  
Systemic Metastasis ◽  
Poorly Differentiated ◽  
The Right ◽  
Pterygoid Muscles

Abstract A case report: The patient was a 32-year-old man with diplopia. He was diagnosed as sphenoid sinusitis on MRI by a local doctor and visited an otolaryngologist. MRI showed extensive extension of neoplastic lesions from the clivus to the sphenoid sinus to the anterior ethmoid sinuses, bilateral cavernous sinuses, and the right medial and lateral pterygoid muscles. The right Lebiere’s lymph node was enlarged and thought to be a metastatic site. Based on the rapid growth and extension of the tumor, the patient was referred to the Department of Otolaryngology at our hospital on suspicion of sinonasal carcinoma. The possibility of chordoma could not be denied, so the patient was referred to our department. The patient underwent a joint endoscopic extended transsphenoidal tumor resection. The pathological diagnosis showed mitotic and necrotic features, and the majority of the cells showed highly atypical components without mucous substrate. However, brachyury, a marker for chordoma, was diffusely positive, and there was loss of INI1 (SMARCB1) expression. The final diagnosis was poorly differentiated chordoma. Postoperatively, the tumor in the right cavernous sinus grew rapidly, and the right eye became blind due to obstruction of the superior ophthalmic vein. The patient was treated with Gamma Knife as soon as possible in the hope of local control by high-dose irradiation, and after a total of three irradiations, the residual tumor shrank markedly and symptoms improved, but systemic metastasis occurred in a short period of time and the patient died. The number of cases of poorly differentiated chordoma has been reported rarely (more than 50), and it is more common in children and even rarer in adults. We report this case with a review of the literature.

scholarly journals ETMR-07. ETANTR: A RARE TUMOR IN A RESOURCE-LIMITED SETTING

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii324-iii324
Author(s):  
Julieta Hoveyan ◽  
Manushak Avagyan ◽  
Anna Avagyan ◽  
Ruzanna Papyan ◽  
Samvel Iskanyan ◽  
...  
Keyword(s):  
Survival Rates ◽  
Tumor Resection ◽  
Large Mass ◽  
Intrathecal Methotrexate ◽  
High Dose ◽  
Ongoing Research ◽  
Resource Limited ◽  
The Right ◽  
Immunohistochemical Analyses ◽  
Limited Setting

Abstract INTRODUCTION Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a rare aggressive brain tumor with low survival rates. There are about 80 cases reported in literature since 2000 when it was first described. There is no standard treatment scheme for ETANTR yet. CASE REPORT: A 2 years old boy presented with a month-long of headache and inability to hold his head. CT scan and MRI revealed a large mass in the right frontal lobe with midline shift. Subtotal tumor resection was done. Histological and immunohistochemical analyses was consistent with ETANTR in one laboratory and PNET in another. The second opinion suggested by the Center of Pediatric Oncology, Hematology and Immunology in Moscow the diagnosis ETANTR was confirmed. Taking into account certain similarities with medulloblastoma was decided to provide treatment according to HIT-2014 protocol. Control MRI done after 2 cycles of Block SKK Carboplatin/Etoposide found tumor progression and for that reason patient underwent second surgical resection. Considering the age of the child radiation therapy was not expedient and the decision was to continue treatment with HIT 2014 intensified regimen, which includes Cisplatin, Vincristine, Etoposide, Cyclophosphamide and intravenous High dose Methotrexate with intrathecal Methotrexate. Aiming to evaluate the effectiveness of treatment we are planning to perform MRI after this 2nd cycle of intensified regimen. DISCUSSION There are difficulties in diagnosis of rare types of cancers in Armenia. Since there is no approved treatment for ETANTR, there is a need for ongoing research to improve its prognosis.

Residual Tumor Resection After High-Dose Chemotherapy in Patients With Relapsed or Refractory Germ Cell Cancer

2004 ◽  
Vol 22 (18) ◽  
pp. 3713-3719 ◽  
Author(s):  
O. Rick ◽  
C. Bokemeyer ◽  
S. Weinknecht ◽  
J. Schirren ◽  
T. Pottek ◽  
...  
Keyword(s):  
Germ Cell ◽  
Mature Teratoma ◽  
Univariate Analysis ◽  
Cell Cancer ◽  
Tumor Resection ◽  
Residual Tumor ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Incomplete Resection ◽  
Residual Tumor Resection

Purpose To assess the role of residual tumor resection performed after high-dose chemotherapy (HDCT) in patients with relapsed or refractory germ cell tumors (GCT). Patients and Methods Between July 1987 and October 1999, postchemotherapy resections of residual tumors were performed in 57 patients who had been treated with HDCT for relapsed or refractory GCT and who had achieved a partial remission to this treatment. Results Complete resections of residual masses were achieved in 52 (91%) of 57 patients who were rendered disease free; in five (9%) of 57 patients, the resections were incomplete. Resection of a single site was performed in 39 (68%) of 57 patients, and the remaining 18 (32%) of 57 patients required interventions at two or more residual tumor sites. Necrosis was found in 22 (38%) of 57 patients, mature teratoma with or without necrosis was found in nine (16%) of 57 patients, and viable cancer with or without additional necrosis or mature teratoma was found in 26 (46%) of 57 patients. Viable cancer consisted either of residual germ cell or undifferentiated cancer in 22 (85%) of 26 patients, with additional non-GCT histologies in the remaining four patients. Patients with viable cancer had a significantly inferior outcome after surgery compared with patients with necrosis and/or mature teratoma even if all cancer was completely resected. Pulmonary lesions with a diameter of more than 2 cm were the only predictive variable for viable cancer in univariate analysis. Conclusion Resections of all residual tumors should be attempted in patients with relapsed or refractory GCT and partial remissions after HDCT.

scholarly journals Endoscope-assisted contralateral transmaxillary approach to the clivus and the hypoglossal canal: technical case report

2019 ◽  
Vol 130 (5) ◽  
pp. 1609-1615 ◽  
Author(s):  
Eva Pamias-Portalatin ◽  
Deependra Mahato ◽  
Jordina Rincon-Torroella ◽  
Tito Vivas-Buitrago ◽  
Alfredo Quiñones-Hinojosa ◽  
...  
Keyword(s):  
Tumor Resection ◽  
Cranial Nerves ◽  
Inferior Turbinate ◽  
Residual Tumor ◽  
Anatomical Location ◽  
Endoscopic Endonasal ◽  
Petroclival Region ◽  
Proton Beam Radiotherapy ◽  
Hypoglossal Canal ◽  
The Right

Clival lesions are still considered surgically complex due to their anatomical location. Critical structures, such as the internal carotid arteries (ICAs), cavernous sinuses, cranial nerves, and brainstem, may be encased within the lesion. Although advances in endoscopic endonasal approaches have provided new routes to these lesions, exposure and resection of clival tumors through the endonasal route remain a technical challenge. Here, the authors report a left-sided endoscopic transmaxillary approach to access the right aspect of the clivus and the hypoglossal canal.A 35-year-old woman presented with progressive right 6th cranial nerve palsy. MRI revealed a contrast-enhancing right petroclival chondrosarcoma that involved Meckel’s cave and extended into the right hypoglossal canal. An endoscopic-contralateral-transmaxillary approach through a left sublabial incision was used to access the right petroclival region and right hypoglossal canal. A left maxillary osteoplastic flap was elevated to expose the left maxillary sinus. This was followed by a left medial maxillectomy, gaining access to the left posterior nasal cavity. The posterior third of the left inferior turbinate and nasal septum were removed to access the right side of the petroclival region. Near-total resection was achieved without any vascular or neurological complications. A thin shell of residual tumor was left behind due to involvement of vital structures, such as the ICA, and further treated with proton-beam radiotherapy.The endoscopic-contralateral-transmaxillary approach provides a direct surgical corridor and good lateral visualization of the skull base vasculature. This approach allows wide maneuverability around the ICA and hypoglossal canal, which, in this case, allowed maximal tumor resection with full preservation of neurological function.

Chondromyxoid Fibroma of the Skull Base: Our Experience with an Elusive Disease

2017 ◽  
Vol 78 (06) ◽  
pp. 576-581 ◽  
Author(s):  
Giancarlo D'Andrea ◽  
Guido Trasimeni ◽  
Venceslao Wierzbicki ◽  
Veronica Picotti ◽  
Alessandra Serraino ◽  
...  
Keyword(s):  
Skull Base ◽  
Local Control ◽  
Osteolytic Lesion ◽  
Tumor Resection ◽  
Brain Magnetic Resonance Imaging ◽  
High Dose ◽  
Adjuvant Radiation ◽  
Chondromyxoid Fibroma ◽  
Histologic Diagnosis ◽  
The Right

Chondromyxoid fibroma (CMF) is an extremely rare lesion of the skull base. This histologic type typically predilects metaphysis of the long bones. It is locally invasive/infiltrative, and this tendency is more concerning in the skull base, where a radical resection is often technically impossible because of the presence of vital neurovascular structures. We present a case of a 19-year-old woman who presented with a sudden onset of right facial weakness, progressively worsening to a severe disfiguring motor weakness. Gadolinium-enhanced brain magnetic resonance imaging showed an osteolytic lesion located in the right mastoid involving the stylomastoid foramen and the right seventh cranial nerve. A partial mastoidectomy was performed, with an excellent rate of tumor resection and complete local control of the disease at follow-up. The analysis of current literature indicates that a radiologic diagnosis is rarely strictly convincing of CMF. Histologic diagnosis is often difficult due to the lack of a specific immunohistochemical pattern of chondrosarcoma. Surgery is currently recognized as the mainstay to manage this lesion, although a trend toward adjuvant radiation therapy (RT) currently is seen. Although a tendency of local recurrence is well recognized in the literature, the very slow and indolent behavior of this lesion plus the trend to enhance local control of the disease with high-dose RT pushed us to a reappraise the role of radical skull base surgeries burdened by the risks of major complications, cosmetic deformities, and additional neurologic deficits.

scholarly journals Advanced lung cancer in combination with liver echinococcosis

2021 ◽  
Vol 25 (2) ◽  
pp. 124-132
Author(s):  
A. B. Egorkina ◽  
Yu. A. Stepanova ◽  
G. G. Karmazanovsky ◽  
D. V. Kalinin ◽  
A. V. Zhao
Keyword(s):  
Lung Cancer ◽  
Diagnostic Errors ◽  
Final Diagnosis ◽  
Advanced Lung Cancer ◽  
Surgical Operation ◽  
Radiation Imaging ◽  
Poorly Differentiated ◽  
Distant Spread ◽  
The Right ◽  
Immunohistochemical Studies

Presented clinical case is a rare case of poorly differentiated squamous cell non-keratinizing cancer of the right lung with ingrowth into the diaphragm and liver, metastatic liver damage in combination with recurrent echinococcosis of the liver. Taking into account the epidemiological history, the surgery, the features of radiation imaging and the results of serology in the preoperative period, the diagnosis of “echinococcosis of the liver with spread to the lungs” was made, which turned out to be incorrect. The diagnosis during the surgical operation was changed, but also turned out to be incorrect. The final diagnosis was made only on the basis of histological and immunohistochemical studies.The reasons of diagnostic errors are analyzed, the emphasis is made on the criteria of differential diagnosis. Typical features of visualization of liver echinococcosis, lung cancer with local and distant spread are presented.

scholarly journals Malignant Hemangioendothelioma in an Irradiated Rat

1972 ◽  
Vol 9 (6) ◽  
pp. 471-474 ◽  
Author(s):  
A. Talerman
Keyword(s):  
Blood Cells ◽  
Lateral Side ◽  
High Dose ◽  
External Radiation ◽  
X Ray ◽  
Local Spread ◽  
Adnexal Tumor ◽  
Poorly Differentiated ◽  
Male Hybrid ◽  
The Right

A male hybrid F1 rat developed a tumor on the lateral side of the right thigh, which had been exposed to a high dose of external radiation (3,500 rads), administered as a single dose by an X-ray machine 16 months previously. The tumor grew rapidly and showed extensive local spread, but there were no metastases at autopsy. The tumor was ulcerated, dark red and hemorrhagic. It was not encapsulated and invaded the surrounding muscles extensively. Microscopically the tumor was composed of anastomozing channels lined by swollen anaplastic endothelial cells that were either heaped up and projected into the lumen or were free within the lumen. Mitotic activity was evident. There were also more solid areas as well as large cavities containing blood cells. The tumor was diagnosed as a malignant hemangioendothelioma. Seven other rats exposed at the same time to various doses of external radiation developed tumors at the site of exposure. There were four fibrosarcomas, one anaplastic, poorly differentiated connective tissue tumor, one squamous cell carcinoma, and one adnexal tumor of skin.

Extracorporeal irradiation of tumorous calvaria

2000 ◽  
Vol 93 (3) ◽  
pp. 494-497 ◽  
Author(s):  
Matthew A. Manning ◽  
Robert M. Cardinale ◽  
Rupert K. Schmidt-Ullrich ◽  
Brian D. Kavanagh ◽  
Isaac L. Wornom ◽  
...  
Keyword(s):  
Tumor Resection ◽  
Residual Tumor ◽  
Frontal Bone ◽  
High Dose ◽  
Content Type ◽  
Prosthetic Implants ◽  
Cadaveric Transplantation ◽  
Extracorporeal Irradiation ◽  
Very High ◽  
Fine Print

✓ This patient with recurrent meningioma grossly involving the frontal bone underwent craniotomy and tumor resection. During the procedure a bone flap was irradiated extracorporeally at a very high dose (120 Gy) sufficient to sterilize residual tumor cells, and the bone was then successfully replaced orthotopically for reconstruction. The use of autologous irradiated bone in this setting offers advantages over cadaveric transplantation and prosthetic implants. Radiation might cause less disruption of the bone's architecture than other techniques of tumor cell eradication.

scholarly journals Regressed Seminoma with Metastases to the Jejunum and Retroperitoneum

2021 ◽  
pp. 418-423
Author(s):  
Nozomi Kashu ◽  
Shoji Oura ◽  
Naoki Kataoka ◽  
Shinichiro Makimoto
Keyword(s):  
Differential Diagnosis ◽  
Lymph Nodes ◽  
Tumor Cells ◽  
Residual Tumor ◽  
Final Diagnosis ◽  
Undifferentiated Carcinoma ◽  
Pathological Examination ◽  
Left Testis ◽  
Regional Lymph Nodes ◽  
Poorly Differentiated

A 66-year-old man with vomiting and weight loss was referred to our hospital. Abdominal computed tomography showed small bowel obstruction caused by a presumed small intestinal tumor. Single-balloon endoscopy showed an ulcerated tumor and marked stenosis of the jejunum. Immunohistochemical staining suggested the tumor to be poorly differentiated or undifferentiated carcinoma. The patient underwent open surgical resection of the jejunal tumor and regional lymph nodes both to improve the quality of life of the patient and to possibly get a cure of the presumed jejunal carcinoma. Pathological examination of the excised tumor and lymph nodes including para-aortic lymph nodes showed large-sized tumor cells and massive lymphocyte infiltrates. Immunostaining showed the tumor cells to be OCT3/4, AE1/AE3, CD117, and D2-40 positive, leading to the diagnosis of metastatic seminoma. With the preoperative diagnosis of a presumed burned-out tumor of the testis, the patient underwent left high orchiectomy. Pathological examination of the left testis showed marked scar tissue, no teratoma elements, and no residual tumor cells. Under the final diagnosis of regressed seminoma, the patient has received combination chemotherapy using bleomycin, etoposide, and cisplatin as adjuvant chemotherapy. Surgical oncologists should take regressed seminoma into their differential diagnosis when the biopsy specimens of the presumed intestinal malignancy show poorly differentiated or undifferentiated atypical cells with massive lymphocyte infiltrates, especially in postpubertal men. Confirmation of a malignant noninvasive component should be another important clue to the appropriate differential diagnosis when choosing between metastatic seminoma and poorly differentiated or undifferentiated intestinal primary malignancies.

Eosinophilic vasculitis: an inhabitual and resistant manifestation of a vasculitis

VASA ◽  
2010 ◽  
Vol 39 (4) ◽  
pp. 344-348 ◽  
Author(s):  
Jandus ◽  
Bianda ◽  
Alerci ◽  
Gallino ◽  
Marone
Keyword(s):  
Skin Biopsy ◽  
Intravenous Iron ◽  
Small Vessel Vasculitis ◽  
High Dose ◽  
Raynaud Phenomenon ◽  
The Third ◽  
Left Elbow ◽  
Right Hand ◽  
The Right ◽  
Trunk Skin

A 55-year-old woman was referred because of diffuse pruritic erythematous lesions and an ischemic process of the third finger of her right hand. She was known to have anaemia secondary to hypermenorrhea. She presented six months before admission with a cutaneous infiltration on the left cubital cavity after a paravenous leakage of intravenous iron substitution. She then reported a progressive pruritic erythematous swelling of her left arm and lower extremities and trunk. Skin biopsy of a lesion on the right leg revealed a fibrillar, small-vessel vasculitis containing many eosinophils.Two months later she reported Raynaud symptoms in both hands, with a persistent violaceous coloration of the skin and cold sensation of her third digit of the right hand. A round 1.5 cm well-delimited swelling on the medial site of the left elbow was noted. The third digit of her right hand was cold and of violet colour. Eosinophilia (19 % of total leucocytes) was present. Doppler-duplex arterial examination of the upper extremities showed an occlusion of the cubital artery down to the palmar arcade on the right arm. Selective angiography of the right subclavian and brachial arteries showed diffuse alteration of the blood flow in the cubital artery and hand, with fine collateral circulation in the carpal region. Neither secondary causes of hypereosinophilia nor a myeloproliferative process was found. Considering the skin biopsy results and having excluded other causes of eosinophilia, we assumed the diagnosis of an eosinophilic vasculitis. Treatment with tacrolimus and high dose steroids was started, the latter tapered within 12 months and then stopped, but a dramatic flare-up of the vasculitis with Raynaud phenomenon occurred. A new immunosupressive approach with steroids and methotrexate was then introduced. This case of aggressive eosinophilic vasculitis is difficult to classify into the usual forms of vasculitis and constitutes a therapeutic challenge given the resistance to current immunosuppressive regimens.

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