Understanding esophageal neurofibroma: A case series and systematic review.

INTRODUCTION: Esophageal neurofibroma is a rare benign esophageal neoplasm. With very few cases documented in the literature, not much is known about the demographics and clinicopathologic features of this tumor. This study was aimed at presenting a case report of an esophageal neurofibroma, and to conduct a systematic review of published cases. METHOD: This review was performed according to the PRISMA guidelines. Literature search was conducted through PubMed, SCOPUS, and Cochrane Databases from inception until May 2020 for all histologically confirmed cases of esophageal neurofibroma. RESULTS: 28 cases, including the newly reported case, were included in the review. The mean age at diagnosis was 53.3 years ±12.1. 53.6% were male. Dysphagia was the most common presenting symptom (53.6%). Most of the reported cases involved the upper esophagus (39.3%). The most utilized diagnostic test was esophagogastroduodenoscopy (57.1%). The mean tumor size was 6.1 cm ± 5.1. Preoperative biopsy was done for 9 cases, out of which seven were negative or inconclusive. In 17 cases (60.7%), immunohistochemical (IHC) staining of the resected tumor was not performed. S100 was the most utilized IHC stain. Enucleation (39.3%) was the most common treatment, followed by esophagectomy (28.6%). CONCLUSION: Esophageal neurofibroma should be considered in the setting of dysphagia caused by a subepithelial tumor. Accurate preoperative histologic diagnosis by using a well-defined biopsy algorithm, in conjunction with IHC analysis, will favor less aggressive surgical treatment and surveillance of asymptomatic lesions. Minimally invasive surgical treatment is feasible and should be considered when the expertise is available.

Typical carcinoids, goblet cell carcinoids, mixed adenoneuroendocrine carcinomas, neuroendocrine carcinomas and adenocarcinomas of the appendix: a comparative analysis of survival profile and predictors.

BACKGROUND: Goblet cell carcinoids (GCC) and other atypical neuroendocrine tumors (NET) of the appendix as well as appendiceal adenocarcinoma are usually managed with the same algorithm as colon cancers. This study investigates clinicopathological features that are predictive of survival outcomes for appendiceal NET and adenocarcinoma. Survival profiles for the histologic subtypes of appendiceal NET and adenocarcinoma were compared. METHODS: A retrospective review of appendiceal NET and adenocarcinoma for patients who are 18 years and above in the SEER database from 2010 to 2014. RESULTS: Females outnumbered males in a 1.3 to 1 ratio in the NET subgroup and 1.1 to 1 ratio in the adenocarcinoma group. The mean age at diagnosis for all NET was 50.3±17 years while that of adenocarcinomas was 60.8±14.1 years. Within the NET subgroups, the mean ages for typical carcinoids (TC), GCC, NEC and MANEC were 42.9±17.3, 56.7±13.7, 45.6±17.4 and 59.7±12.8 years, respectively. Overall survival for adenocarcinoma was 86.3%, 73.5%, 65.7%, and 57.6% for 1-, 2-, 3- and 4-year OS, respectively. For NET, TC showed better survival profile with 1- and 4-year overall survival of 97.4% and 95.7%, respectively while MANEC had the worst survival outcome with 1- and 4-year OS of 88.6% and 62.2%, respectively. GCC had a better 1-year OS compared to NEC (95.5% versus 92.9%) but showed slightly worse 4-year OS (82% versus 84.8%). Age at diagnosis (HR 1.03), African-American race (HR 1.47) and stage IV disease (HR 9.58) were independent predictors of survival for appendiceal adenocarcinoma. For NET, advanced age at diagnosis, advanced disease stage and the African-American race were identified as negative independent predictors of survival. CONCLUSIONS: While prior studies have suggested that atypical NET (GCC, NEC and MANEC) are more likely to present at more advanced stages, this study showed that most cases of GCC, MANEC and NEC were diagnosed at stages I and II. Appendiceal adenocarcinoma, on the other hand, presented mostly at stage IV. With respect to OS, atypical histologic subtypes of NET have worse outcome compared to TC. Although better OS was noted for GCC, NEC and MANEC when compared to adenocarcinoma, this benefit was lost in stage IV disease where adenocarcinoma recorded better 1- and 4-year OS. Prospective and randomized studies which provide granular details of treatment are needed to better define treatment algorithm for appendiceal NET.

A rare combination of giant right retroperitoneal schwannoma and right adrenal oncocytoma as an incidental finding.

Adrenal oncocytomas and retroperitoneal schwannomas are two groups of very rare tumors with distinct histologic features. Both tumors are usually incidentally found and are mostly benign. Optimal management is usually with surgical resection. We report a unique case of a 74-year-old woman who was incidentally found to have these two exceptionally rare tumors at the same time. She was successfully managed with surgical resection of the retroperitoneal schwannoma and adrenalectomy for the adrenal oncocytoma and both diagnoses were confirmed with histopathology and immunochemistry.

A case report of an incidental Brenner tumor found after resection of a large ovarian mucinous neoplasm.

INTRODUCTION: Brenner Tumors are rare adenofibromas that are most commonly benign and discovered in post-menopausal women. PRESENTATION OF CASE: This is a case report of a 57-year-old female with three months of progressively worsening abdominal pain due to a large abdominal mass discovered on CT scan. Surgical removal of the mass revealed a giant mucinous tumor of the ovary with an associated Brenner tumor that was discovered incidentally. DISCUSSION: Although the Brenner tumor was accurately identified in the intraoperative frozen section evaluation, the mucinous tumor was underdiagnosed by frozen section as benign when permanent section revealed borderline mucinous cystadenoma. This finding did not change the treatment course for this particular patient as she had expressed personal preference for total abdominal hysterectomy. However, underdiagnosis of frozen sections of ovarian tumors is not rare. It is unclear whether an associated Brenner tumor increases malignancy potential. CONCLUSION: Further investigation is required to determine whether associated Brenner tumors found during frozen section are more highly associated with malignancy and could therefore change intraoperative and overall decision making.

Pleomorphic hyalinizing angiectatic tumor arising in the groin: A case report.

INTRODUCTION: Pleomorphic hyalinizing angiectatic tumors are a rare group of tumors that are currently classified as benign tumors of unknown differentiation. To our knowledge, less than 100 cases have been reported in literature. We report a case that presented in the groin - an uncommon location for this rare tumor. CASE REPORT: A 75 year-old female presented with a seven-year history of painless right groin mass with rapid growth of 2 year duration. On physical examination, a firm and mobile mass was identified in the right groin. It measured 12cm×8cm, with no clinically palpable lymph nodes. Microscopic and immunohistochemical features were consistent with pleomorphic hyalinizing angiectatic tumor. DISCUSSION: Pleomorphic hyalinizing angiectatic tumor is a rare soft tissue tumor usually diagnosed using microscopic and immuohistochemical analysis to allow for differentiation from other soft tissue tumors. It is treated by wide local excision.