An alternative therapeutic procedure for sinonasal undifferentiated carcinoma

We describe two cases of sinonasal undifferentiated carcinoma, a rare and highly aggressive neoplasm of the paranasal sinuses. Case 1 had first been diagnosed as esthesioneuroblastoma, and intra-arterial and systemic chemotherapy and radiotherapy were performed. The tumor became smaller but did not disappear, and the patient died 2 months later. Case 2 received systemic chemotherapy and chemoradiotherapy, with daily venous administration of cisplatin during radiotherapy. The tumor became much smaller but also remained, and the patient died a month later. Although the therapy failed, chemoradiotherapy has some possibility of controlling the disease. Received: 15 February 2001 / Accepted: 10 April 2001     

Minimally invasive endoscopic resection of sinonasal undifferentiated carcinoma

Purpose

The purpose of the study was to review a single-institution experience with endoscopic resection of sinonasal undifferentiated carcinoma (SNUC).

Materials and Methods

Thirteen patients underwent treatment of SNUC between January 2002 and July 2009. Retrospective data were collected including demographics, tumor characteristics, surgical strategy, adjuvant therapies, local and regional recurrence, distant metastasis, overall survival, and disease-free survival.

Results

The mean age was 51.8 years. The most common tumor stage at presentation was T4 (92%). Seven patients (53%) were treated with minimally invasive endoscopic resection (MIER) with negative intraoperative margins. Endoscopic anterior skull base resection was performed in 5 patients, and endoscopic-assisted bifrontal craniotomy was performed in 1 patient to clear the superior tumor margin. Six patients received pre- or postoperative chemoradiation. One patient underwent palliative chemoradiation, and one patient underwent open craniofacial resection. In the MIER group, simultaneous local and regional recurrence was observed in 1 patient (14%) after 30 months. Distant metastases were observed in 2 other patients (28%) without local or regional recurrence. All 3 patients with recurrences died of their disease. The remaining 4 patients were clinically, endoscopically, and radiographically free of disease, resulting in overall and disease-free survival rates of 57% with mean follow-up of 32.3 months.

Conclusions

These preliminary data suggest a potential role for MIER in the comprehensive management algorithm of SNUC in appropriately selected patients. Patient outcomes including local and regional recurrence, distant metastases, and overall and disease-free survival were comparable to a treatment strategy using traditional craniofacial resection.

Level of evidence

2b.     

Improved clinical outcomes with multi-modality therapy for sinonasal undifferentiated carcinoma of the head and neck

ObjectiveTo examine outcomes among patients treated for sinonasal undifferentiated carcinoma (SNUC) of the head and neck.Study designRetrospective review.MethodsThe records of 16 consecutive patients with newly diagnosed, non-metastatic SNUC were analyzed. Initial treatment consisted of: surgery alone (6 patients), surgery with post-operative chemoradiotherapy (4 patients), and primary radiation therapy with concurrent chemotherapy (6 patients).ResultsThe median survival for patients treated by surgery followed by postoperative chemoradiotherapy was 30 months compared to 7 months and 9 months for patients treated by surgery alone and upfront chemoradiotherapy, respectively (p = 0.20). The 2-year locoregional control was 18% for patients treated with upfront chemoradiotherapy, 37% for patients treated with surgery alone, and 78% for patients treated with surgery plus chemoradiotherapy (p = 0.49).ConclusionWhile the potential role of selection bias must be considered, multi-modality therapy using surgery and post-operative chemoradiotherapy yielded the most favorable outcomes for SNUC and should be recommended whenever feasible.     

Esthesioneuroblastoma and sinonasal undifferentiated carcinoma: impact of histological grading and clinical staging on survival and prognosis

OBJECTIVES: Hyams proposed a histological grading system for esthesioneuroblastoma in which grade I tumors have an excellent prognosis and grade IV tumors are uniformly fatal. The Hyams grading system predated advanced craniofacial techniques, extensive use of immunohistochemistry, and the recognition of sinonasal undifferentiated carcinoma (SNUC) as a distinct entity. Therefore we aimed to determine whether Hyams classification is useful in predicting outcome for esthesioneuroblastoma and SNUC. STUDY DESIGN: A retrospective review of cases from 1970 to 1999. METHODS: Twenty-six patients (12 with esthesioneuroblastoma and 14 with SNUC) were reviewed. The Kadish clinical stage was determined, and histopathological slides were reviewed and graded using the Hyams system. RESULTS: Kadish staging was available for 26 patients (2 patients with stage A tumors; 7 with stage B; and 17 with stage C). Of the 8 evaluable patients with Kadish stage A or B tumors, 6 remained disease free for more than 2 years compared with only 5 of the 17 Kadish stage C tumors. Slides were available for Hyams grading in 21 patients (2 patients with grade I tumors; 4 with grade II; 4 with grade III; and 11 with grade IV). Of the 6 patients with Hyams grade I or II tumors, 4 remained disease free for more than 2 years compared with only 4 of the 15 patients with Hyams grade III or IV tumors. Of note, three patients with Kadish stage C tumors (two with esthesioneuroblastoma, one with SNUC) and two patients with Hyams grade IV tumors (one with esthesioneuroblastoma and one with SNUC) survived for more than 5 years. CONCLUSIONS: Both the Hyams grading system and the Kadish staging system can be used as independent predictors of outcome. Although limited by small numbers, the results of this study demonstrate that patients with either advanced clinical stage or pathological grade of esthesioneuroblastoma or SNUC have poor prognosis, but that long-term survival is possible in these patients if aggressive treatment is used.     

Combined-modality treatment improved outcome in sinonasal undifferentiated carcinoma: single-institutional experience of 21 patients and review of the literature

The optimal treatment of sinonasal undifferentiated carcinoma (SNUC) remains unclear. We report our results on the outcome and toxicity of patients with SNUC treated by a combined modality and attempt to define the optimal treatment strategies by reviewing the literature. Between 1996 and 2010, 21 consecutive patients with SNUC were treated by any combination of surgery, chemotherapy and radiotherapy. End points were local control (LC), regional control (RC), disease-free (DFS), cause-specific (CSS) overall survival (OS), and late toxicity. Organ preservation was defined as visual preservation without orbital exenteration. After median follow-up of 54 months, the 5-year actuarial rates of LC, RC, DFS, CSS, and OS were 80, 90, 64, 74, and 74 % respectively. On multivariate analysis, T-stage and multimodality treatment approach correlated significantly with LC. Elective nodal irradiation was given to 42 % of high-risk node-negative patients. None of them developed regional failure. The overall 5-year incidence of grade ≥2 late toxicity was 30 %. Treatment-related blindness was significantly decreased in patients treated with intensity-modulated radiotherapy (IMRT), compared to 2D and 3D-conformal radiotherapy (3DCRT), with organ preservation rates of 86 and 14 % respectively (p = 0.006). We concluded that combined-modality treatment with three, or at least two, modalities resulted in good LC, but with high overall rate of late toxicity. However, the incidence of late toxicity and permanent visual impairment were decreased over time by the introduction of IMRT. Because of the improvement in therapeutic ratio achieved by using IMRT, this highly conformal radiation technique should be the standard of care in patients with SNUC.     

Human papillomavirus-related multiphenotypic sinonasal carcinoma

Human papillomavirus-related multiphenotypic sinonasal carcinoma (HMSC) is a rare primary tumor of the sinonasal tract that has been reported recently. It is reportedly associated with human papillomavirus infection. The tumor presents with glandular cyst-like histology, but some cases exhibit squamous epithelialization and are positive on p16 immunohistochemical staining. The clinical picture and treatment of this disease have not been established. However, this report describes a recurrent case of this disease treated with salvage surgery. The patient was a 61-year-old woman who presented to the previous doctor with a chief complaint of nasal obstruction. A localized tumor was found in the left nasal cavity and was resected under endoscopic guidance. The postoperative pathological diagnosis was HMSC. Twenty-one months after the surgery, local recurrence was observed, and the patient was referred to our hospital. Since the recurrent lesion had widely infiltrated outside the nasal cavity, extensive resection and reconstructive surgery were performed. Postoperative radiotherapy was performed as an additional treatment. Notably, 13 months have passed since the salvage surgery, and no recurrence has been observed to date.     

Outcomes for endoscopic resection of sinonasal squamous cell carcinoma

OBJECTIVES: Preliminary data suggest efficacy for the endoscopic approach for sinonasal malignancy. The purpose of this study is to evaluate the effectiveness of the endoscopic approach for the resection of sinonasal squamous cell carcinoma (SCC). STUDY DESIGN: Retrospective analysis of patients undergoing endoscopic resection of sinonasal SCC from August 1996 to May 2004. METHODS: Eleven patients were treated with curative intent. Demographic data, histopathology, extent of tumor involvement, and need for adjunctive radiation or chemotherapy were determined. Local recurrence (LR), distant metastasis, overall survival (OS), and disease-free survival (DFS) rates were calculated. RESULTS: The mean age for the patient population was 62.5 (52-85) years with a male:female ratio of 8:3. Multimodality therapy including radiation or chemotherapy was used in 8 of 11 (73%) patients. Seven patients were resected using strictly an endoscopic approach, whereas four required combined endoscopic and neurosurgical resection. LR and distant metastatic rates were 20% and 0%, respectively. OS and DFS were both 91%, with mean follow-up of 31.5 (range 6-88) months. CONCLUSIONS: Endoscopic resection in combination with multimodality therapy is an effective method for curative resection of sinonasal SCC. In this preliminary study, it facilitated complete resection with acceptable LR and DFS rates.     

鼻腔鼻窦肌上皮癌临床分析

目的 探讨鼻腔鼻窦肌上皮癌的临床病理特点、治疗方法 及疗效.方法 回顾性分析四川省人民医院和浙江大学医学院附属第一医院1990年1月～2008年12月经病理确诊的鼻腔鼻窦肌上皮癌7例患者的临床资料.结果 7例患者均行手术治疗,随访6个月～7年,6例术后复发.3例术后出现颈淋巴结转移,其中2例行颈淋巴结清扫术,1例无手术...     

Radiotherapy and chemotherapy for nasopharyngeal carcinoma

NPC is unique among head and neck cancers, in that it is radiocurable and moderately chemosensitive. Modern diagnostic techniques have facilitated tumor definition, with consequent improvement in staging accuracy and radiotherapy planning. Our standard radiotherapy technique incorporates routine prophylactic neck irradiation of early tumors. Special techniques for more advanced tumors are described. Neoadjuvant cis-platinum-containing chemotherapy has proved useful in controlling regional and distant metastatic disease.     

Sinonasal undifferentiated carcinoma: an update

PURPOSE OF REVIEW: Sinonasal undifferentiated carcinoma is a rare and extremely malignant tumor of the paranasal sinuses. Historically, treatment outcomes have been poor. This review presents recent data on the management of sinonasal undifferentiated carcinoma and examines treatment trends that may result in improved locoregional control and survival. RECENT FINDINGS: Patients who receive aggressive multimodality treatments have improved outcomes. In particular, a chemoradiotherapy regimen including concurrent platinum-based chemotherapy given preoperatively or postoperatively to patients with resectable disease seems to result in better disease-free survival. Neoadjuvant chemotherapy, although strongly advocated by some, is currently not offered by many. Although surgery seems to be an important part of the treatment for sinonasal undifferentiated carcinoma, its ideal timing, either upfront or after radiotherapy, remains uncertain. SUMMARY: Overall, outcomes for sinonasal undifferentiated carcinoma are poor. An aggressive approach using surgery, platinum-based chemotherapy, and radiation seems to offer the greatest chance for significant locoregional control and survival.     

Salivary duct carcinoma in the sinonasal tract

Salivary duct carcinoma (SDC) is an uncommon malignant tumor, characterized by aggressive behavior and poor prognosis. SDC usually arises from ductal epithelium of the major salivary glands, and it is quite infrequent elsewhere. We present a rare case of a 73-year-old man with SDC, which is possibly originated from the paranasal sinuses or the lacrimal system. Microscopic evaluation revealed that the tumor cells, with pleomorphic nuclei and abundant eosinophilic cytoplasm, formed cell nests and duct-like structure. A cribriform growth pattern was also seen. Immunohistochemical staining was positive for cytokeratins (CAM 5.2 and 34βE12), gross cystic disease fluid protein 15 (GCDFP-15), and androgen receptor protein, while p63 and involucrin were negative. The patient already had multiple metastasis of the tumor in the lung at diagnosis, and he could not undergo definitive surgical procedures, because of severe restrictive lung disease. Although SDC in the sinonasal tract is quite rare, SDC should be in the differential diagnosis in these regions, due to its aggressive behavior and poor prognosis.     

Postirradiated neuroendocrine carcinoma of the sinonasal tract

OBJECTIVES/HYPOTHESIS: Neuroendocrine carcinoma (NEC) of the sinonasal tract is rare. In this present study, we report our treatment experience with sinonasal NEC. STUDY DESIGN: Retrospective case series. METHODS: A retrospective review of the clinical outcomes and pathology of 18 patients with NEC arising from the sinonasal region. RESULTS: Ten tumors were primary NEC without previous radiation, and eight tumors were postirradiated NEC occurring within the radiation field for previous nasopharyngeal carcinoma in six patients and tonsillar lymphoma in one and neck metastasis of unknown primary origin in one, with an interval between previous radiotherapy and diagnosis of NEC from 82 to 385 months, with a mean of 197 months. Fifteen tumors were small cell carcinoma, two were atypical carcinoid, and one was typical carcinoid tumor. Fifteen patients underwent surgery with/without postoperative adjuvant chemoradiotherapy. Three patients received induction chemotherapy or primary radiotherapy with further definitive treatment. The 5-year disease-free and overall survival rates of all 18 patients were 56.1% and 62.2%, respectively. In comparing primary NEC with postirradiated NEC, they were similar in age, sex distribution, stage, pathology, and treatment, and the 5-year overall survival rates were 70% and 62.5%, respectively. CONCLUSIONS: In this series, postirradiated NEC is common, which may be the result of there being a large number of long-term nasopharyngeal carcinoma survivors. The prognoses of postirradiated NEC and primary NEC appear to be similar despite the relatively short follow-up period in the postirradiated NEC group.     

Large-cell undifferentiated carcinoma of the submandibular gland

Large-cell undifferentiated carcinoma (LCUC) arising in the submandibular gland is so rare that there have been only a few reported cases. We encountered a 65-year-old Japanese male, whose left submandibular gland was enlarged due to LCUC. Exenteration of the left submandibular gland together with selective neck dissection was performed, followed by postoperative radiotherapy. Current literature concerning the histopathological and clinical features of this neoplasm was reviewed.     

Endonasal endoscopic surgery for sinonasal squamous cell carcinoma from an oncological perspective

Endonasal endoscopic surgery (EES) has been applied to the management of sinonasal (SN) tumors based on recent advances in endoscopic surgical techniques and technologies over the past three decades. EES has been mainly indicated for benign tumors and less aggressive malignant tumors. Notwithstanding this, EES has been gradually adopted for squamous cell carcinoma (SCC), which is the most common histology among SN malignancies. However, an analysis of the outcomes of EES for patients with SCC is difficult because most articles included SCC a wide range of different tumor histologies. Therefore, we herein review and clarify the current status of EES focusing on SCC from an oncological perspective.The oncologic outcomes and the ability to achieve a histologically complete resection are similar between endoscopic and open approaches in highly selected patients with SN-SCC. Surgical complications associated with EES are likely similar for SN-SCC compared to other sinonasal malignancies.The indications for a minimally invasive approach such as EES in the management of patients with SN-SCC should be stricter than those for less aggressive malignant tumors because of the aggressive nature of SCC. Also, it is important to achieve negative surgical margins with EES in patients with SCC. We believe that the indications for EES for SN-SCC are widening due to advances in diagnostic imaging, and endoscopic surgical techniques and technologies. However, while expanding the indications for EES for SN-SCC we must carefully confirm that the outcomes support this strategy.     

Lymphoepithelioma (undifferentiated carcinoma) of the laryngohypopharynx

Lymphoepithelioma (World Health Organization type 3, undifferentiated carcinoma of the nasopharyngeal type) is an extremely rare cancer in the laryngohypopharyngeal regions. We found reports of only 6 such tumors in the English language medical literature; 1 of the 6 was from our institution. We report four additional cases seen at the Mayo Clinic. Radiotherapy controlled disease at the primary site in three of the four cases. The neoplasm has a propensity for early, widespread dissemination. Three of our four patients died of extensive visceral and nodal metastases. One patient has survived eight years without a recurrence.     

The role of endoscopic resection for selected patients with sinonasal squamous cell carcinoma

ObjectiveDespite of rapid advances in endoscopic surgery, the gold standard for sinonasal squamous cell carcinoma (SNSCC) surgery has remained the open approach with en-block resection due to the aggressive nature of SNSCC, including frequent recurrence and high mortality rate. For that reason, few studies have focused on SNSCC treated by endoscopic surgery alone. The objective of this study was to evaluate the usefulness of endoscopic surgery for patients with SNSCC.MethodsA retrospective analysis was performed for 15 consecutive SNSCC patients who underwent endoscopic surgery without an open approach. We carefully selected patients whose tumor attachment sites could be fully visualized and completely resected through an endonasal approach.ResultsOf the fifteen patients, 4 patients (27%) were diagnosed with T1, 7 (47%) with T2, 4 (27%) with T3, and no patients with T4a or T4b disease. Four of the 15 (27%) patients showed positive surgical margins. The 5-yr overall survival, disease-specific survival, and local control rate was 72.4%, 79.6%, and 92.9%, respectively. The 5-yr disease-specific survival for T1, T2, and T3 disease was 100% and 75% and 75%, respectively. Patients with negative surgical margins had a better disease-specific survival rate than did those with positive surgical margins (p = 0.0253).ConclusionEndoscopic surgery for patients with SNSCC appears to afford an effective method in selected cases. The achievement of negative surgical margins with a good view of the tumor attachment site was considered to be critical to the management of SNSCC.