Radiologic overview of sinonasal lesions.

Sinonasal tumors are often malignant and comprise approximately 3% of all head and neck malignancies. Half of these tumors arise in the nasal cavity, and other common locations of origin include the ethmoid and maxillary sinuses. Some unique clinical features are anosmia and altered phonation but the most common general features include headache, epistaxis, and diplopia. CT and MRI may be used to assess tumor location, invasion of adjacent tissue, presence of metastasis, internal tumor heterogeneity, and contrast enhancement. Local invasion of the tumor beyond the sinonasal tract can impact adjacent structures such as the cranial nerves, skull base, branches of the internal carotid artery, and orbit leading to neurologic signs, facial pain, and diplopia. Imaging is used in the diagnosis, staging, and treatment planning of sinonasal tumors. This collection of benign and malignant sinonasal tumors will include some rare and unique cases with an emphasis on imaging features demonstrating a wide variety of pathologies.

A case of resected anaplastic carcinoma of the pancreas producing granulocyte-colony stimulating factor with literature review.

BACKGROUND: Granulocyte colony-stimulating factor (G-CSF)-producing tumors have been reported in various organs, and the prognosis of patients with G-CSF-producing pancreatic cancers is particularly dismal. In this report, we present a case of G-CSF-producing anaplastic carcinoma of the pancreas (ACP), characterized by early postoperative recurrence and rapid, uncontrolled growth. CASE PRESENTATION: A 74-year-old man presented to our hospital with complaints of abdominal fullness and pain after eating. On admission, it was observed that the peripheral leukocyte counts and serum G-CSF levels were significantly elevated (23,770/µL and 251 pg/mL, respectively). Computed tomography of the abdomen revealed a pancreatic head tumor involving the superior mesenteric vein. Pathologically, ultrasound-guided fine-needle aspiration confirmed ACP. Subsequently, we performed a subtotal stomach-preserving pancreaticoduodenectomy with portal vein reconstruction and partial transverse colon resection. On postoperative day (POD) 7, the leukocyte count decreased from 21,180/µL to 8490/µL; moreover, computed tomography revealed liver metastasis. Therefore, mFOLFILINOX chemotherapy was initiated on POD 30. However, the tumor exhibited rapid progression, and the patient died on POD 45. CONCLUSIONS: G-CSF-producing ACP is rare, and the prognosis of patients is extremely poor. Basic research is required to develop effective drugs against G-CSF-producing tumors, and large-scale studies using national databases are needed to develop multidisciplinary treatment methods.

AB018. It's not always olfactory neuroblastoma-sinonasal undifferentiated carcinoma with frontal base and orbital involvement, a rare disease with poor prognosis: a case report.

BACKGROUND: Malignant neoplasms of the paranasal sinuses and nasal cavity are rare, comprising only 3% of all head and neck malignancies. Sinonasal undifferentiated carcinoma is a rare malignancy and an aggressive neoplasm that was clinic-pathologically distinct from other poorly differentiated malignancies of the nasal cavity and sinuses. The lesion is thought to originate from the epithelium, grows rapidly and invades nearby structures, often resulting in bony destruction while rapid progression of symptoms over weeks to months is characteristic. CASE DESCRIPTION: A 75-year-old man initially presented to ophthalmologist due to blurry vision and diplopia for 2 months. There was also progressive right eye swelling and pain for the past 12 months. Further image study revealed a right superomedial orbital mass with frontal/ethmoid sinus and frontal base extension, causing significant right eye gaze limitation, visual loss, ptosis, chemosis and exophthalmos. He was then transferred to neurosurgical clinic for further treatment where combined surgery was advocated to relieve the mass effect and also for pathology proof. Orbital roof/medial wall and frontal skull base were invaded and destroyed by the tumor. A bicoronal craniotomy was performed and extensive tumor removal was achieved except the orbital fossa due to difficult separation from the ocular muscle and optic nerve. Frontal base was reconstructed in a layered fashion to avoid cerebrospinal fluid (CSF) leakage and infection. He was sent to intensive care unit (ICU) for overnight observation where no further neurologic deterioration occurred. Instant orbital pain relief was noted with significantly decreased proptosis and improving visual acuity. Detailed pathological staining and molecular tests revealed a sinonasal undifferentiated carcinoma, adjuvant chemotherapy was arranged, followed by 30 cycles of radiotherapy. Slight disease progression was noted 12 months after the surgery, chemotherapy regimen was adjusted according to clinical response and he is still currently under regular adjuvant treatment. CONCLUSIONS: Sinonasal undifferentiated carcinoma typically carries a poor prognosis despite aggressive medical and surgical treatment, as it always presents at an advanced stage. Different combinations of chemotherapy, radiotherapy, radical surgical resection have been used to improve the outcome yet there is still not a universal treatment strategy. Early diagnosis, prompt treatment, and comprehensive medical care are crucial to achieve the best possible outcome for affected patients.

The role of radiotherapy in the treatment of sinonasal undifferentiated carcinoma: A population-based analysis.

BACKGROUND: Sinonasal undifferentiated carcinoma (SNUC) is a rare, aggressive disease with ambiguous management and poor prognosis. This study aimed to evaluate the role of radiation therapy (RT) and explore the optimal treatment sequence. METHODS: Retrospective analysis of survival trends of 410 SNUC patients between 1973 and 2015. RESULTS: The 5-year cancer-specific survival (CSS) rate (45.1%) and overall survival (OS) rates (38.1%) were reported in the 84-month median follow-up. Radiotherapy was a prognosticator for improving CSS (hazard ratio [HR] = 0.425, 95% confidence interval [CI]: 0.299-0.603, p = 0.000) and OS (HR = 0.415, 95% CI: 0.303-0.570, p = 0.000), either with surgery (p = 0.000) or without surgery (p = 0.000). However, in a combined therapy of surgery and RT, preoperative and postoperative RT (5-year OS rates were 47.1% and 45.6%, respectively, p = 0.486) were not significantly different. CONCLUSIONS: Radiotherapy plays a key role in improving SNUC survival rates. No significant difference in survival rates was observed in preoperative and postoperative RT treatment.

Contemporary treatment and outcome of sinonasal undifferentiated carcinoma: A meta-analysis.

Induction chemotherapy (IC) recently gained importance for treatment of sinonasal undifferentiated carcinoma (SNUC). We analyzed our SNUC cases and performed a meta-analysis with focus on survival-rates stratified by treatment. SNUC cases at our institution were retrospectively evaluated. A systematic literature review was conducted to analyze treatment and outcome of SNUC. To calculate 5-year and 2-year overall survival (OS), individual patient data (IPD) were analyzed using Kaplan-Meier estimators and Cox proportional hazard regression to identify associations between types of therapy and survival. A random effects model for pooled estimates of 5-year survival was applied to studies without IPD data. Five-year OS of our SNUC cases (n = 9) was 44.4%. The IPD analysis (n = 192) showed a significantly better 5-year OS for patients who received induction chemotherapy (72.6% vs. 44.5%). The pooled 5-year OS of 13 studies identified in the literature search was 43.8%. IC should be considered in every patient diagnosed with SNUC.

A Rare Case of Undifferentiated Carcinoma of the Liver Arising in the Context of Primary Biliary Cholangitis.

Undifferentiated carcinoma of the liver is a rare and difficult-to-detect form of primary liver cancer. We herein report the first case of undifferentiated carcinoma of the liver in a 70-year-old Japanese woman with primary biliary cholangitis. The patient was diagnosed with cStage IVA liver cancer (85 mm in diameter) and treated with hepatic arterial infusion chemotherapy, 30 Gy radiotherapy, and 11 courses of on-demand transarterial chemoembolization. Although the hepatic tumor had markedly shrunk (from 85 to 20 mm), the patient ultimately died 16 months after the diagnosis due to rapid growth of lymph node metastases.

Undifferentiated carcinoma with osteoclast-like giant cells of the pancreas: a narrative review.

Undifferentiated carcinoma with osteoclast-like giant cells of the pancreas (UCOGCP) is a rare pancreatic tumor that accounts for less than 1% of all pancreatic malignancies. The characteristic pathological manifestation of UCOGCP is the presence of osteoclast-like giant cells (OGCs) distributed among pleomorphic undifferentiated tumor cells. UCOGCP can occur either alone or in association with other types of pancreatic tumors. At present, there is no unified consensus or guideline for the diagnosis and treatment of UCOGCP, and most of the literature are individual case reports. With the accumulation in the number of clinical cases and the development of precision medicine technology, the understanding of UCOGCP is also deepening. Researchers have begun to recognize that UCOGCP is a pancreatic tumor with distinctive clinical and molecular characteristics. In this review, we focus on the latest research status and future exploration directions in the diagnosis, treatment, and prognosis of UCOGCP.

Outcomes of SWI/SNF complex-deficient sinonasal carcinomas in a Southeast Asian cohort.

BACKGROUND: SWI/SNF complex-deficient sinonasal carcinomas are rare, genetically distinct, and aggressive entities. METHODS: SMARCB1 and SMARCA4 immunohistochemistry was retrospectively performed on a cohort of undifferentiated, poorly differentiated, and poorly defined sinonasal carcinomas. Survival outcomes were compared between SMARCB1/SMARCA4 (SWI/SNF complex)-deficient and -retained groups. RESULTS: Eight SWI/SNF complex-deficient (six SMARCB1-deficient, two SMARCA4-deficient) cases were identified among 47 patients over 12 years. Triple-modality treatment was more frequently utilized in SWI/SNF complex-deficient carcinomas than in SWI/SNF complex-retained carcinomas (71.4% vs. 11.8%, p = 0.001). After a median follow-up of 21.3 (IQR 9.9-56.0) months, SWI/SNF complex-deficient sinonasal carcinomas showed comparable recurrence rates (57.1% vs. 52.9%, p = 0.839), time-to-recurrence (7.3 [IQR 6.6-8.3] vs. 9.1 [IQR 3.9-17.4] months, p = 0.531), and overall survival (17.7 [IQR 11.8-67.0] vs. 21.6 [IQR 8.9-56.0] months, p = 0.835) compared to SWI/SNF complex-retained sinonasal carcinomas. CONCLUSION: Triple-modality treatment may improve survival in SWI/SNF complex-deficient sinonasal carcinomas.

An overview of the contemporary diagnosis and management approaches for anaplastic thyroid carcinoma.

Thyroid carcinoma is a complex disease with several types, the most common being well-differentiated and undifferentiated. The latter, "undifferentiated carcinoma", also known as anaplastic thyroid carcinoma (ATC), is a highly aggressive malignant tumor accounting for less than 0.2% of all thyroid carcinomas and carries a poor prognosis with a median survival of 5 months. BRAF gene mutations are the most common molecular factor associated with this type of thyroid carcinoma. Recent advances in targeted biological agents, immunotherapy, stem cell therapy, nanotechnology, the dabrafenib/trametinib combination therapy, immune checkpoint inhibitors (ICI) and artificial intelligence offer novel treatment options. The combination therapy of dabrafenib and trametinib is the current standard treatment for patients with BRAF-V600E gene mutations. Besides, the dabrafenib/trametinib combination therapy, ICI, used alone or in combination with targeted therapies have raised some hopes for improving the prognosis of this deadly disease. Younger age, earlier tumor stage and radiotherapy are all prognostic factors for improved outcomes. Ultimately, therapeutic regimens should be tailored to the individual patient based on surveillance and epidemiological data, and a multidisciplinary approach is essential.

Histogenetic insights and genetic landscape of fibromatosis-like undifferentiated gastric carcinoma: a focused study.

BACKGROUND: The aim of this study was to elucidate the histogenesis and genetic underpinnings of fibromatosis-like undifferentiated gastric carcinoma (FLUGC), a rare pathological entity. METHOD: Through a detailed analysis of seven cases, including histopathological evaluation, CTNNB1 gene mutation screening, human epidermal growth factor receptor 2 (HER2) protein level quantification, and HER2 gene amplification assessment to identify the pathological and molecular characteristics of FLUGC. RESULTS: Of the seven patients in this study, five were male and two were female (age: 39-73 years). Four patients presented with lesions in the gastric antrum and three had lesions in the lateral curvature of the stomach. Histopathologically, over 90% of the tumor consisted of aggressive fibromatosis-like tissue, including proliferating spindle fibroblasts and myofibroblasts and varying amounts of collagenous fibrous tissues. Undifferentiated cancer cells, accounting for less than 10%, were dispersed among the aggressive fibromatosis-like tissues. These cells were characterized by their small size and were relatively sparse without glandular ducts or nested mass-like structures. Immunophenotyping results showed positive expression of CKpan, CDX2, villin, and p53 in undifferentiated cancer cells; positive expression of vimentin in aggressive fibromatosis-like tissue; positive cytoplasmic expression of ß-catenin; and focal cytoplasmic positive expression of smooth muscle actin (SMA). Genetic analysis did not reveal any mutations in the CTNNB1 gene test, nor was there amplification in the HER2 gene fluorescence in situ hybridization (FISH) test. Additionally, the Epstein-Barr encoding region (EBER) of in situ hybridization was negative; and the mismatch repair (MMR) protein was positive. Programmed cell death-1 (PD-1) was < 1-5%; programmed cell death ligand 1 (PD-L1): TPS = 1-4%, CPS = 3-8. CONCLUSION: The study highlights the significance of CTNNB1, HER2, EBER, and MMR as pivotal genetic markers in FLUGC, underscoring their relevance for diagnosis and clinical management. The rarity and distinct pathological features of FLUGC emphasize the importance of accurate diagnosis to prevent underdiagnosis or misdiagnosis and to raise awareness within the medical community.