Survival in Sinonasal Melanoma: A Meta-analysis

Sinonasal melanoma is an uncommon tumor which carries a poor prognosis and high rates of local and regional recurrence and distant metastasis. While surgical resection is the mainstay of treatment, the utility of multimodality therapy has not been well studied or established. We sought to better evaluate the optimal treatment modality for sinonasal melanoma. We reviewed 39 case reports involving 423 patients with sinonasal melanoma and present a meta-analysis comparing survival by treatment modality. The two-tailed p-value for survival by treatment modality was determined. The number of primary site/local, regional, and distant recurrences was determined where data was available. There was a nonsignificant increase in survival for patients treated with surgery + radiotherapy versus surgery alone. There was a statistically significant increase in survival for surgery + chemotherapy versus chemotherapy alone and versus surgery alone. Patients treated with combined surgery, radiation, and chemotherapy had a statistically shorter survival interval than patients treated with surgery + chemotherapy, which may reflect more advanced disease in patients treated with triple therapy. There was no statistically significant increase in survival found for the addition of radiation to surgery. This meta-analysis demonstrates that multimodality therapy, particularly the addition of chemo-or immunotherapy to surgery, may increase survival in a subset of patients. Radiation therapy did not appear to increase survival. There may be a significant increase in overall survival with combined modality therapy with surgery and chemo/immunotherapy versus single modality therapy. Level of evidence: III. Grade of recommendation: C.     

Sinonasal paraganglioma: endoscopic resection with a 4-year follow-up

BACKGROUND: Paragangliomas are uncommon neuroendocrine tumors. In the head and neck, they are most commonly seen at the carotid body, the temporal bone, or along the vagus nerve. Sinonasal paragangliomas are extremely rare. Most cases reported describe management by open surgical resection, with sublabial and/or transfacial approaches. METHODS: We present a case of a large sinonasal paraganglioma successfully managed by endoscopic resection. RESULTS: The patient has no evidence of recurrence after 4 years of follow-up. CONCLUSIONS: This case demonstrates that sinonasal paragangliomas might be amenable to endoscopic resection. However, the most important priority to consider when selecting a surgical technique is whether adequate excision with clear margins can be obtained. Furthermore, because of the aggressive nature of these tumors, regardless of the surgical approach, long-term follow-up is recommended.     

Experience with mucosal melanoma of the nose and paranasal sinuses

Purpose: Sinonasal mucosal melanoma is a rare and aggressive disease and its incidence does not mimic that of its cutaneous counterpart in the Australian population. The present study examines one unit's experience with the disease and proposes a treatment strategy. The significance of macroscopic widespread mucosal melanosis and histological melanoma in situ is considered in the present study to be crucial in overall survival and the main cause of local failure and is specifically addressed. Methods: The present study represents the retrospective experience of the multidisciplinary Head and Neck Clinic at the Prince of Wales Hospital over a 30‐years period (from 1970 to end 1999) in the management of the disease, including both primary and salvage treatment approaches. The study includes 27 patients treated with surgery with or without postoperative radiation therapy. Management of recurrence was also considered. Results: The mean time to local recurrence was 14.7 months and the mean time to distant metastases was 23.2 months. Mean survival time was 52 months and mean time from local recurrence to death was 75 months. Overall, disease free and disease specific survival and survival post‐recurrence were analysed by the Kaplan?Meir method. A cancer specific 5 years survival of 46% was achieved, which compares favourably with recent international series. Conclusion: Sinonasal mucosal melanoma remains an aggressive disease with the possibility of local recurrence years after initial treatment, however, initial radical surgery encompassing the primary lesion and distant in situ or satellite disease and postoperative radiotherapy can offer long‐term control, as can reoperation for local recurrence where appropriate.     

Anorectal Melanoma Misdiagnosed as Hemorrhoids: A Case Report and Review of the Literature

Anorectal melanoma is a rare and aggressive malignant neoplasm with an indolent course, manifesting with nonspecific symptoms and a poor prognosis. We present a case of anorectal melanoma that was initially treated as hemorrhoids and correctly diagnosed after lower gastrointestinal endoscopy. We also present the latest findings in the literature about anorectal melanomas and discuss updates about treatment options and management.     

Primary Sinonasal Mucosal Melanoma with Aberrant Diffuse and Strong Desmin Reactivity: A Potential Diagnostic Pitfall!

The broad morphologic spectrum, inherent immunophenotypic heterogeneity of malignant melanoma and its rarity in the sinonasal tract are major challenges in eliciting the correct diagnosis, which may lead to misclassification and inadequate medical management. Herein, we describe a single case of a 70 year-old male with sinonasal mucosal melanoma, exhibiting varying histologic phenotypes including small round blue cell morphology, epithelioid and focal rhabdoid morphology and strong, diffuse desmin immunoreactivity. These constellation of features initially prompted the diagnosis of rhabdomyosarcoma. The differential diagnosis in this anatomic area includes other malignant small round blue cell tumors of the sinonasal mucosa such as rhabdomyosarcoma, olfactory neuroblastoma, sinonasal undifferentiated carcinoma, and lymphoma. We reviewed precedent literature and further discuss the potential pitfalls to which pathologists may be prone.     

Oral Amelanotic Melanoma: A Systematic Review of Case Reports and Case Series

Oral amelanotic melanoma (OAM) is a rare, non-pigmented mucosal neoplasm representing less than 2% of all melanoma. The present study analyses the available data on OAM and describes its clinicopathological features, identifying potential prognostic factors. Online electronic databases such as PubMed-Medline, Embase, and Scopus were searched using appropriate keywords from the earliest available date till 31st March 2021 without restriction on language. Additional sources like Google Scholar, major journals, unpublished studies, conference proceedings, and cross-references were explored. 37 publications were included for quantitative synthesis, comprising 55 cases. The mean age of the patients was 59.56 years, and the lesions were more prevalent in males than in females. OAM’s were most prevalent in the maxilla (67.2%) with ulceration, pinkish-red color, nodular mass, and pain. 2 patients (3.36%) were alive at their last follow-up, and 25 were dead (45.4%). Univariate survival analysis of clinical variables revealed that age older than 68 years (p = 0.003), mandibular gingiva (p = 0.007), round cells (p = 0.004), and surgical excision along with chemotherapy & radiation therapy (p = 0.001) were significantly associated with a lower survival rate. Oral Amelanotic Melanoma is a neoplasm with a poor prognosis, presenting a 6.25% possibility of survival after 5 years. Patients older than 68 years, lesions in the mandibular gingiva, round cells, and surgical excision along with chemotherapy and radiotherapy, presented the worst prognosis. However, they did not represent independent prognostic determinants for these patients.Supplementary InformationThe online version contains supplementary material available at 10.1007/s12105-021-01366-w.     

Merkel Cell Carcinoma: 30-Year Experience from a Single Institution

Background

Merkel cell carcinoma (MCC) is a rare cutaneous malignancy. Few single-institution series have been reported.

Methods

Review of MCC patients treated at our institution between 1980 and 2010. Patient, tumor, and treatment variables were analyzed to determine MCC-specific outcomes.

Results

We identified 161 patients with MCC. There was a 2.5-fold increase in cases over the last decade. Median length of follow-up was 36 months. Stage at diagnosis was I in 35 %, II in 21 %, IIIa in 12 %, IIIb in 23 %, and IV in 9 %. The 5-year MCC-specific survival rates were 87, 63, 42, and 0 % for stages I, II, III, and IV, respectively. Death from the disease occurred in 10 % of patients with T1 and in 50 % with larger lesions. One-third of patients presented with nodal disease. Sentinel lymph node biopsy (SLNB) identified micrometastases in 9 out of 27 (33 %) early-stage patients. Recurrence developed in 56 % of SLNB-positive and 39 % of SLNB-negative patients. Half of patients recurred after a median time of 9 months. Proportions of first recurrence location were distant (52 %), nodal (27 %), and local (21 %). Adjuvant treatments did not improve recurrence or survival rates. One-third of patients died of the disease.

Conclusions

SLNB identifies micrometastasis in one-third of early-stage patients. Negative SLNB may predict for improved but not necessarily favorable outcome. Initial tumor size and clinical nodal disease predict for poor outcome. High recurrence rates warrant the development of more effective adjuvant therapies, and better markers of recurrence and treatment response for MCC are needed.     

Long-Term Survivals of Stage IIB Osteosarcoma: A 20-Year Experience in a Single Institution

Background

The purpose of this study is to evaluate the disease-free survival (DFS) and overall survival (OS) of patients with stage IIB osteosarcoma at a single institution for 20 years and to compare the results according to the chemotherapy protocols.

Methods

From Jan 1988 to Nov 2008, 167 patients with osteosarcoma were treated at our hospital and among them, 117 patients (67 males and 50 females) with stage IIB osteosarcoma were evaluable. Their mean age was 22.6 years (range, 8 months to 71 years). Seventy-eight cases underwent the modified T10 (M-T10) protocol (group 1), 23 cases underwent the T20 protocol (group 2) and 16 cases underwent the T12 protocol (group 3). The DFS and OS were calculated and compared according to the chemotherapy protocols.

Results

At a mean follow-up of 78.9 months, 63 patients were continuously disease-free (63/117), 6 patients were alive after having metastatic lesions, 7 patients died of other cause and 41 patients died of their disease. The 5- and 10-year OS rates were 60.2% and 44.8%, respectively and the 5- and 10-year DFS rates were 53.5% and 41.4%, respectively. There was no significant difference of the OS and DFS between the chemotherapy protocols (p = 0.692, p = 0.113).

Conclusions

At present, we achieved success rates close to the internationally accepted DFS and OS. We were able to achieve the higher survival rates using the M-T10 protocol over the 20 years. However, there was no significant difference of results between the chemotherapy protocols. We think the M-T10 protocol will achieve more favorable results in the near future.     

Outcomes Following Isolated Limb Infusion for Melanoma. A 14-Year Experience

Background  Isolated limb infusion (ILI) is a minimally invasive technique for delivering regional chemotherapy in patients with advanced and metastatic melanoma confined to a limb. It is essentially a low-flow isolated limb perfusion (ILP) performed via percutaneous catheters without oxygenation. Methods  From our prospective database 185 patients with advanced metastatic melanoma of the limb treated with a single ILI between 1993 and 2007 were identified. In all patients a cytotoxic drug combination of melphalan and actinomycin-D was used. Drug circulation time was 20–30 min under mild hyperthermic conditions (38–39°C). Results  The majority of patients (62%) were female. Their average age was 74 years (range 29–93 years). Most patients had MD Anderson stage III disease (134/185). The overall response rate was 84% [complete response (CR) rate 38%, partial response rate 46%]. Median response duration was 13 months (22 months for patients with CR; P = 0.01). Median follow-up was 20 months and median survival was 38 months. In those patients with a CR, the median survival was 53 months (P = 0.005). CR rate and survival time decreased with increasing stage of disease. On multivariate analysis significant factors for a favorable outcome were achievement of CR, stage of disease, thickness of primary melanoma, the CO₂ level in the isolated circuit, and a Wieberdink limb toxicity score of III (considerable erythema and edema). Conclusion  The response rates and duration of response after ILI are comparable to those achieved by conventional ILP. ILI is a minimally invasive alternative to the much more complex and morbid conventional ILP technique for patients with advanced metastatic melanoma confined to a limb.