scholarly journals A Case of Glomangiopericytoma at the Nasal Septum

2017 ◽  
Vol 12 (4) ◽  
pp. 572-575 ◽  
Author(s):  
Takashi Anzai ◽  
Tsuyoshi Saito ◽  
Sho Tsuyama ◽  
Miri Toh ◽  
Katsuhisa Ikeda ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Nasal Septum ◽  
Low Grade ◽  
Beta Catenin ◽  
Mesenchymal Neoplasm ◽  
Resected Tumor ◽  
Vascular Structures ◽  
Borderline Malignancy ◽  
Fibrous Tumor ◽  
The Right

Abstract Glomangiopericytoma (GPC) is a rare sinonasal perivascular tumor that accounts for < 0.5–1% of all sinonasal tumors. GPC is categorized as a low-grade neoplasm with borderline malignancy and a tendency of local recurrence. GPC is a rare mesenchymal neoplasm characterized by the perivascular proliferation of tumor cells, and it requires being distinguished from solitary fibrous tumors. Here, we report a case of GPC in a 68-year-old male patient who presented at the emergency room of our hospital with a complaint of sudden epistaxis. A small, reddish, protruding tumor was observed on the right nasal septum. A biopsy revealed a possible perivascular tumor such as a GPC or solitary fibrous tumor. Thus, we performed complete resection with endoscopic surgery. The size of the resected tumor was 12 × 5 mm, and it showed a uniform proliferation of oval-to-short spindle-shaped cells with slightly branching vascular structures. The tumor cells showed minimal cytologic atypia and there were an average of 3 mitoses in 10 high power fields. Necrosis was not observed. The tumor cells showed strong and diffuse nuclear immunostaining with beta catenin and were negative with STAT6, CD34 and bcl-2. The MIB-1 labeling index was approximately 5%. Genetic testing revealed CTNNB1 mutation (p.S33C). Thus, a diagnosis of low grade GPC was made on the biopsy and the patient could be successfully treated with endoscopic resection.

scholarly journals JAZF1–SUZ12 endometrial stromal sarcoma forming subserosal masses with extraordinary uptake of fluorodeoxyglucose on positron emission tomography: a case report

2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Koto Fujiishi ◽  
Shigenori Nagata ◽  
Rieko Kano ◽  
Chiaki Kubo ◽  
Maasa Shirayanagi ◽  
...  
Keyword(s):  
Positron Emission Tomography ◽  
Endometrial Stromal Sarcoma ◽  
Emission Tomography ◽  
Low Grade ◽  
Neoplastic Cells ◽  
Pelvic Magnetic Resonance Imaging ◽  
Stromal Sarcoma ◽  
Positron Emission ◽  
Mesenchymal Neoplasm ◽  
The Right

Abstract Background Low-grade endometrial stromal sarcoma (ESS) is rare mesenchymal neoplasm, recently specified as harboring JAZF1–SUZ12 rearrangement. Typical JAZF1–SUZ12 ESS is slow growing, in which high uptake of fluorodeoxyglucose (FDG) on positron emission tomography (PET) and subserosal masses are quite unusual. Case presentation A 69-year-old Japanese woman complained of urinary incontinence. Pelvic magnetic resonance imaging showed uterine lesions composed of (1) a 9 × 8 × 7-cm mass protruding from the right-anterior wall, (2) a 4.5-cm mass attached to the right-posterior wall, and (3) a 6.5-cm intramural mass in the fundus. FDG-PET demonstrated maximum standardized uptake value of 13.28 confined to the two subserosal masses (1 & 2) in contrast to no uptake of the intramural mass (3). She was diagnosed with a high-grade uterine sarcoma concomitant with leiomyomas and underwent total hysterectomy with bilateral salpingo-oophorectomy and pelvic lymphadenectomy. The removed uterus had three tumors—two in the right-anterior and right-posterior subserosa, respectively, and the remaining in the fundal myometrium. Microscopically, the three tumors shared morphologic features characterized by neoplastic cells similar to proliferative-phase endometrial stromal cells, in which neither round-cell component, pleomorphism, nor high mitotic activity was recognized. Nuclear cyclin D1 immunostaining was identified 50% of neoplastic cells in the two subserosal tumors (1 &2) whereas < 1% positive cells in the intramural component (3). Reverse transcriptase-polymerase chain reaction showed the same-sized electrophoretic bands indicating JAZF1–SUZ12 gene fusion shared by the three uterine tumors and a focal tumor extension into the extrauterine vein. The patient is alive without evidence of recurrence at 14 months after surgery. Conclusions Pathologists and clinicians should not exclude the possibility of JAZF1–SUZ12 ESS even when uterine subserosal masses demonstrate extraordinary FDG uptake on PET. Molecular analysis is helpful for diagnostic confirmation of JAZF1–SUZ12 ESS with a complex growth pattern.

scholarly journals Keratoacanthoma of the Nasal Septum Secondary to Ranibizumab Use

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Jason E. Cohn ◽  
Hilary M. Caruso Sales ◽  
Giang Huong Nguyen ◽  
Harvey Spector ◽  
Kenneth Briskin
Keyword(s):  
Nasal Septum ◽  
Skin Lesions ◽  
Low Grade ◽  
Cutaneous Lesions ◽  
Epithelial Tumor ◽  
Vegf Inhibitor ◽  
Central Crater ◽  
Well Differentiated ◽  
The Right ◽  
Endothelial Growth Factor

Keratoacanthoma (KA) is a benign epithelial tumor that typically presents as a firm, cone-shaped, flesh-colored nodule with a central horn-filled crater. KA is considered to be a low-grade variant of squamous cell carcinoma (SCC). We report a rare case of a 72-year-old male who presented with a KA involving the nasal septum, possibly related to ranibizumab use. A flesh-colored lesion on the right anterior nasal septum lesion was visualized on examination. Histologic examination revealed a well-circumscribed, dome-shaped central crater filled with keratin, well-differentiated squamous epithelium with ground-glass cytoplasm with pushing margins, and intraepithelial microabscesses establishing the diagnosis of KA. KA of the nasal septum has only been reported once in the literature. This case is unusual because it normally presents on sun-exposed areas. Additionally, this patient was taking ranibizumab, a vascular endothelial growth factor (VEGF) inhibitor for macular degeneration. Despite ranibizumab not being directly linked to precancerous and cancerous skin lesions, agents in this medication class have been. Although it is difficult to prove associations in this isolated case, the role of ranibizumab causing cutaneous lesions should be further investigated.

scholarly journals Primary sinonasal renal cell-like adenocarcinoma: a case report and review of the literature

2020 ◽  
Author(s):  
Xiaoxia Gou ◽  
Yanzhe Wang ◽  
Fang Chen ◽  
Xiaoli Liu ◽  
Peigang Ruan ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Renal Cell ◽  
Low Grade ◽  
Cell Renal Cell Carcinoma ◽  
Ki 67 ◽  
Pathological Characteristics ◽  
S 100 ◽  
Clear Cytoplasm ◽  
The Right ◽  
Irregular Mass

Abstract BackgroundPrimary sinonasal renal cell-like adenocarcinoma (SNRCLA) is an extremely rare neoplasm with unique clinical and pathological features. At present, there is no summary of its clinical and pathological characteristics. We treated one case and reported to here. Review the domestic and foreign literature, summarize its clinical pathological characteristics and diagnosis and treatment.CaseA 69-year-old female presented with repeated epistaxis, nasal obstruction of 2-years’ duration. Computed tomography (CT) was performed revealing an irregular mass of the right nasal cavity involving choana and nasopharynx. The patient was treated with endonasal endoscopic resection. Histologically, SNRCLA was very similar to clear cell renal cell carcinoma (RCC), the tumor cells were round or polygonal in size with abundant clear cytoplasm and uniform nuclei. The tumor cells positive for CK7, Vimentin, EMA, S-100, Ki-67 (5% +) and negative for CEA, P53, P63 by immunohistochemical staining,. The patient is free from recurrence over 27 months after the surgery.ConclusionSNRCLA seems to be a low-grade localized carcinoma associated with favorable prognosis. Surgical resection is recommended as the main stay of treatment.

scholarly journals Wide Excision of a Retroperitoneal Liposarcoma with En Bloc Ureterectomy and Renal Salvage by Autotransplantation

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Siegfredo R. Paloyo ◽  
Arjel D. Ramirez ◽  
Ferri P. David-Paloyo ◽  
Rodney B. Dofitas
Keyword(s):  
The Philippines ◽  
Wide Excision ◽  
Low Grade ◽  
En Bloc ◽  
Excellent Outcome ◽  
Mesenchymal Neoplasm ◽  
Renal Autotransplantation ◽  
Characteristic Features ◽  
Well Differentiated ◽  
The Right

Liposarcoma is a malignant mesenchymal neoplasm composed of adipose tissue with varying degrees of atypia. These tumors grow slowly and may reach an enormous size particularly if located in the retroperitoneum. We report a 40-year-old male with a 6-month history of gradual abdominal enlargement. Computed tomography (CT) of the abdomen showed a huge retroperitoneal mass with characteristic features consistent with liposarcoma. On laparotomy, the mass was noted to be encasing the right ureter for which a wide excision with en bloc ureterectomy and subsequent renal autotransplantation for organ preservation was done. Post-operative course was uneventful with excellent outcome after 6 months of follow-up. Final histopathologic diagnosis was low-grade, well differentiated liposarcoma, which has favorable prognosis following radical surgery. This was the first report of such a case in the Philippines.

scholarly journals Superficial CD34-positive fibroblastic tumor: report of 18 cases of a distinctive low-grade mesenchymal neoplasm of intermediate (borderline) malignancy

2013 ◽  
Vol 27 (2) ◽  
pp. 294-302 ◽  
Author(s):  
Jodi M Carter ◽  
Sharon W Weiss ◽  
Konstantinos Linos ◽  
David J DiCaudo ◽  
Andrew L Folpe
Keyword(s):  
Low Grade ◽  
Mesenchymal Neoplasm ◽  
Borderline Malignancy

Identification Of Strn-Ntrk2 Rearrangement In A High Grade Sarcoma, With Good Clinical Response To Firstline Larotrectinib Therapy

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S79-S79
Author(s):  
R Lin ◽  
Z Wang ◽  
W Jiang ◽  
A Basu-Mallick
Keyword(s):  
Clinical Response ◽  
Day Treatment ◽  
Symptomatic Relief ◽  
Gluteus Medius ◽  
Fusion Partner ◽  
Gene Fusions ◽  
Good Clinical Response ◽  
Mesenchymal Neoplasm ◽  
Fibrous Tumor ◽  
The Right

Abstract Casestudy Gene fusions involving tropomyosin receptor kinase genes, NTRK (NTRK1-3), are important in tumorigenesis. Larotrectinib, a selective NTRK inhibitor, is recently approved to treat NTRK fusion positive solid tumors. We herein report a case of soft tissue sarcoma harboring two STRN-NTRK2 gene fusions, with good clinical response to firstline larotrectinib treatment. Results A 35 year-old female presented with pain in the right gluteal region, and a large solid mass without overlying erythema, edema and induration was identified. Initial MRI study showed a heterogenous, vascular and partially necrotic mass (16.5 x 12.9 x 10.4 cm) centered in the right gluteus medius and maximus muscles. A core biopsy of the mass showed a cellular mesenchymal neoplasm with round/ovoid cells, high mitosis (21 per 10 HPFs) and focal staghorn type vessels, reminiscent of solitary fibrous tumor. However, STAT6 immunostaining was negative. Additional immunostains show no specific lineage. Our in-house NGS fusion panel showed two in-frame STRNNTRK2 fusions, containing the same 5’ partner sequence (exon 1-3) of STRN, with the 3’ fusion partner starting from either the exon 15 or the exon 16 of NTRK2. Due to the large size and location of the tumor, larotrectinib was initiated as firstline therapy. The patient noticed a quick amelioration of tumor related pain, and a significant shrinkage of the size of tumor following the initial 7-day treatment. On post-treatment day 52, MRI showed the tumor significantly decreased in size to 7.7 x 7.4 x 6.6 cm with satisfactory symptomatic relief. Conclusion NTRK2 fusions are relatively rare when compared with NTRK1 and NTRK3, especially in sarcoma. Of note, the only other report in the literature of NRTK2 fusion- positive sarcoma also showed SFT-like morphology, and the patient responded well to larotrectinib as second line therapy.

scholarly journals Olfactory Preservation in Craniofacial Resection of Tumor Invading Hemianterior Skull Base: Operative Video

2021 ◽  
Author(s):  
Kenya Kobayashi ◽  
Yasuji Miyakita ◽  
Fumihiko Matsumoto ◽  
Go Omura ◽  
Satoko Matsumura ◽  
...  
Keyword(s):  
Skull Base ◽  
Nasal Septum ◽  
Cribriform Plate ◽  
Cerebrospinal Fluid Leakage ◽  
Low Grade ◽  
High Grade ◽  
Craniofacial Resection ◽  
Grade Malignancy ◽  
The Right ◽  
High Grade Malignancy

AbstractIn traditional craniofacial resection of tumors invading the anterior skull base, the bilateral olfactory apparatus is resected. Recently, transnasal endoscopy has been used for olfactory preservation in resections of unilateral low-grade malignancies. However, for tumors that invade the orbita or for high-grade malignancies, the transnasal endoscopic skull base surgery has been controversial. This video demonstrates the surgical techniques of olfactory preservation during craniofacial resection of a high-grade malignancy invading the hemianterior skull base and orbita.We present the case of a 32-year-old woman with osteosarcoma in the right ethmoid sinus. The tumor invaded the ipsilateral cribriform plate, dura menta, and orbital periosteum; however, the nasal septum and crista galli were intact (Fig. 1A, B). Because the tumor was a high-grade malignancy and the orbita had been invaded, we performed craniofacial resection instead of endoscopic resection (Fig. C2A). We drilled into the right side of the crista galli, midline of the cribriform plate, and perpendicular plate of the ethmoid bone via craniotomy. As a result, we accessed the nasal cavity directly (Fig. 2B). To preserve the nasal septum, we detached the remaining right septal mucosa through the transfacial approach (Fig. 2C). Because of the high risk of cerebrospinal fluid leakage as a result of previous irradiation, we performed vascularized free flap reconstruction of the skull base instead of pericranial flap.Postoperative computed tomography revealed no evidence of tumor (Fig. 1C, D). The patient's sense of smell returned after 1 postoperative day, and she was discharged on the postoperative day 14.The link to the video can be found at: https://youtu.be/XzPABYwzkjs.

Identification Of Strn-Ntrk2 Rearrangement In A High Grade Sarcoma, With Good Clinical Response To Firstline Larotrectinib Therapy

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S76-S77
Author(s):  
R Lin ◽  
Z Wang ◽  
W Jiang ◽  
A Basu-Mallick
Keyword(s):  
Clinical Response ◽  
Day Treatment ◽  
Symptomatic Relief ◽  
Gluteus Medius ◽  
Fusion Partner ◽  
Gene Fusions ◽  
Good Clinical Response ◽  
Mesenchymal Neoplasm ◽  
Fibrous Tumor ◽  
The Right

Abstract Casestudy Gene fusions involving tropomyosin receptor kinase genes, NTRK (NTRK1-3), are important in tumorigenesis. Larotrectinib, a selective NTRK inhibitor, is recently approved to treat NTRK fusion positive solid tumors. We herein report a case of soft tissue sarcoma harboring two STRN-NTRK2 gene fusions, with good clinical response to firstline larotrectinib treatment. Results A 35 year-old female presented with pain in the right gluteal region, and a large solid mass without overlying erythema, edema and induration was identified. Initial MRI study showed a heterogenous, vascular and partially necrotic mass (16.5 x 12.9 x 10.4 cm) centered in the right gluteus medius and maximus muscles. A core biopsy of the mass showed a cellular mesenchymal neoplasm with round/ovoid cells, high mitosis (21 per 10 HPFs) and focal staghorn type vessels, reminiscent of solitary fibrous tumor. However, STAT6 immunostaining was negative. Additional immunostains show no specific lineage. Our in-house NGS fusion panel showed two in-frame STRN- NTRK2 fusions, containing the same 5’ partner sequence (exon 1-3) of STRN, with the 3’ fusion partner starting from either the exon 15 or the exon 16 of NTRK2. Due to the large size and location of the tumor, larotrectinib was initiated as firstline therapy. The patient noticed a quick amelioration of tumor related pain, and a significant shrinkage of the size of tumor following the initial 7-day treatment. On post-treatment day 52, MRI showed the tumor significantly decreased in size to 7.7 x 7.4 x 6.6 cm with satisfactory symptomatic relief. Conclusion NTRK2 fusions are relatively rare when compared with NTRK1 and NTRK3, especially in sarcoma. Of note, the only other report in the literature of NRTK2 fusion- positive sarcoma also showed SFT-like morphology, and the patient responded well to larotrectinib as second line therapy.

scholarly journals Rare Sinonasal Non-intestinal Type Adenocarcinoma of Right Nasal Septum

2019 ◽  
Vol 3 (1) ◽  
pp. 43
Author(s):  
Adam Mohamad ◽  
Baharudin Abdullah ◽  
Seoparjoo Azmel Mohd Isa ◽  
Norhafizah Abdul Rahim
Keyword(s):  
Nasal Septum ◽  
Young Male ◽  
Intestinal Type ◽  
Low Grade ◽  
Anatomic Structure ◽  
Respiratory Epithelial Adenomatoid Hamartoma ◽  
Seromucinous Hamartoma ◽  
Obstructive Sleep ◽  
Sinonasal Adenocarcinoma ◽  
The Right

Sinonasal tract is a complex anatomic structure with many possible diagnoses. Common diagnoses that usually encountered from the biopsied samples are inflammatory polyps and papillomas as well as squamous cell carcinoma. Another entity which most poorly understood is low grade glandular or tubular proliferations which includes reactive lesions, respiratory epithelial adenomatoid hamartoma, seromucinous hamartoma, and low grade sinonasal adenocarcinoma. We describe a case of a young male with right nasal septum mass which found incidentally upon flexible nasopharyngolaryngoscopic examination, who was referred earlier for possible obstructive sleep apnea. After excision of the tumour was done, it was confirmed to be low grade sinonasal adenocarcinoma non-intestinal type. This case highlights the rarity of the entitiy of low grade sinonasal adenocarcinoma nonintestinal type involving the right nasal septum; and how its subtle finding can be mistaken to be a clinically benign condition.International Journal of Human and Health Sciences Vol. 03 No. 01 January’19. Page : 43-46

scholarly journals SUN-129 Hipoinsulinemic Hipoglycemia Caused by Solitary Fibrous Tumor IGF-2 Producer: Case Report

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Flavia Tedesco Motta ◽  
Thamiris Freitas Maia ◽  
Paulo Enrique Peinado Noriega ◽  
Hially Ribeiro Cabral ◽  
Juliana Farhat ◽  
...  
Keyword(s):  
Solitary Fibrous Tumor ◽  
Oral Prednisone ◽  
Tumor Resection ◽  
Lower Lobe ◽  
Oral Glucose ◽  
Intravenous Glucose ◽  
Breath Sounds ◽  
Mesenchymal Neoplasm ◽  
Fibrous Tumor ◽  
The Right

Abstract BACKGROUND: Solitary Fibrous Tumor is a mesenchymal neoplasm composed of CD34+ fibroblastic cells that can produce spontaneous hypoglycemia by the overproduction of IGF-2. It closely resembles the hypoglycemia characteristic of functioning islet cell tumors. CLINICAL CASE A 77-year-old male was found unconscious and taken to an emergency department with evidence of hypoglycemia and clinical improvement following intravenous glucose administration. He did not have a history of diabetes mellitus and was not taking any glucose lowering medications. He was discharged with nutritional orientation and for control of capillary glycaemia to prevent hypoglycemia. He had 3 episodes of capillary hypoglycemia (50, 45 and 38) at home, that was predominant in the fasting morning and during its occurrence he presented mild sweating, speech difficulty, staring and diplopia, with complete improvement of symptoms after oral glucose replacement. Months earlier, he sought an otolaryngologist for intermittent mild dyspnea; denied cough, hemoptysis, chest pain and unintentional weight loss. He performed chest X-ray with evidence of large right hemithorax mass. Physical examination revealed diminished breath sounds in the right middle and lower lung fields and dullness to percussion. Despite marked hypoglycemia (31 mg/dl), the serum insulin level was less than 0.6  μIU/mL (less than 3 μIU/mL), the C-peptide level was 0.24 nmol/L (less than 0,6 nmol/L), had negative ketonemia and a positive response after glucagon administration (glycaemia increased in 50 mg/dl). Anti-insulin antibodies were negative. Serum cortisol secretion and adrenocorticotropic hormone were normal. The serum level of growth hormone (GH) was 0,03 (less than 0,97ng/ml). The serum IGF-2 level was 227 ng/ml  (267 - 616 ng/ml), the IGF-I level was 72 ng/ml (37,1 - 172 ng/ml) and the IGF2/ IGF1 was 3,15 (equal or greater than 3). Computed tomographic (CT) scan revealed a large heterogeneous mass with dimensions of 17,4 × 15× 12.2 cm. It determines almost total atelectasis of the lower lobe on this side and maintains broad medial contact with the mediastinum, compressing the right atrium and the inferior pulmonary vein on this side. Preoperatively, was administered 40 mg oral prednisone with capillary glucose normalization. The tumor was completely resected and was a grayish-white solid, with dimensions of 17 x 16 x 12 cm. Immunohistochemical stains demonstrated positivity for CD34 and IGF2 expression. Postoperatively, serum glucose and insulin levels returned to normal, and episodes of hypoglycemia are resolved. CONCLUSION This case reinforce the importance of investigate IGF-2 tumor production as a cause of hypoinsulinemic hypoglycemia and reports the complete resolution of hypoglycemia after corticoid administration and/or tumor resection.

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