Intrathyroidal thymic carcinoma.

A young woman in her early 30s presented with a right thyroid mass and progressive hoarseness due to a right vocal cord palsy. The preoperative fine-needle aspiration cytology was classified as Bethesda V and she underwent a total thyroidectomy and neck dissection. Intraoperatively, the thyroid mass was adherent to the oesophagus, trachea and encasing the right recurrent laryngeal nerve which was sacrificed. Final histopathology diagnosed a rare subtype of thyroid cancer known as intrathyroidal thymic carcinoma (ITC). She was then sent for adjuvant radiotherapy after a multidisciplinary tumour board discussion. This case report highlights the difficulty in preoperative diagnosis of ITC and the importance of immunohistochemical staining in clinching the diagnosis. In view of its rarity, there have been no published consensus on the treatment of ITC, hence we would like to share some learning points through a comprehensive literature review.

Omental venous malformation: a rare cause of spontaneous haemoperitoneum.

Spontaneous haemoperitoneum is a rare condition with a variety of aetiologies. Regardless of the cause, it is invariably a life-threatening condition that requires urgent diagnosis and management. The most common causes of spontaneous haemoperitoneum include gynaecological, hepatic, splenic and vascular causes. However, here, we present a rare case of spontaneous haemoperitoneum secondary to idiopathic omental venous malformation.Our patient presented with acute onset of right iliac fossa pain, and a CT scan revealed a massive haemoperitoneum. A laparoscopic converted to open laparotomy was performed to diagnose and treat the patient. We discuss the management approach for such patients with spontaneous haemoperitoneum.

Rare case of pancreatic lipomatous hamartoma.

A middle-aged man had an incidental finding of 10.1 cm lipomatous mass arising from pancreatic body/neck detected on CT scan. He was asymptomatic. He underwent surgical resection of the mass due to concern for malignancy. His postoperative course was complicated by a high-volume pancreatic leak of approximately 900 mL/day. He underwent endoscopic retrograde cholangiopancreatography and insertion of a pancreatic stent, with some improvement in the pancreatic leak. His leak eventually settled after 3 months. The final histopathology showed lobules of mature adipocytes with small islands of disorganised benign pancreatic ducts and acini interspersed within them, suggestive of pancreatic hamartoma of lipomatous variant. Pancreatic lipomatous hamartomas are rare and are often diagnosed on final histopathology when the initial resection was performed due to diagnostic uncertainty or concern for malignancy. It is a benign lesion with an indolent course and must be discriminated from other lipomatous lesions of the pancreas. An awareness of the condition is important to help guide management.

Risk stratification of parotid neoplasms based on intraoperative frozen section and preoperative fine needle aspiration cytology.

PURPOSE: This study aims to determine the relationship of frozen section (FS) to final histology and determine how incorporating FS may change preoperative malignancy risk estimates based on preoperative fine needle aspiration cytology (FNAC). The secondary aim is to determine if FS is useful in influencing intraoperative decision-making. METHODS: Retrospective review of 426 intraoperative FS for parotidectomies performed for primary parotid lesions. RESULTS: Risk of malignancy with a benign FS was 2.5%, with indeterminate 36.1%, and with malignant 100%. Incorporating FS to fine needle aspiration for cytology helped to stratify malignancy risk especially in the Milan categories of atypia of undetermined significance, neoplasm of uncertain malignant potential and non-diagnostic categories, where a malignant FS increased malignancy risk significantly. FS was only able to identify 11% of high-risk histological subtypes for which a neck dissection would be recommended. CONCLUSIONS: FS may be used to stratify malignancy risk intraoperatively but has limited utility in clinical decision-making to perform a neck dissection and more extensive parotid resection in high-risk histological subtypes.

Lymphoepithelial carcinoma of the larynx: an extremely rare tumour in a patient of Chinese descent.

Lymphoepithelial carcinoma (LEC) of the larynx is an extremely rare tumour which, unlike its nasopharyngeal counterpart, has shown a propensity to affect elderly Caucasian men and is not commonly associated with Epstein-Barr virus. We present a 70-year-old Chinese man who complained of hoarseness and dysphagia. Nasoendoscopy revealed a left supraglottic tumour. Preoperative MRI (in particular Diffusion Weighted Imaging) showed the possibility of two distinct components within a tumour. The patient underwent total pharyngolaryngectomy and bilateral selective neck dissection. The final histology report confirmed the presence of a tumour with two distinct components: predominant LEC with a smaller conventional (keratinising) squamous cell carcinoma component. The patient recovered well after surgery and subsequently underwent adjuvant radiotherapy. Final staging was pT3 N2c M0 (AJCC stage IVA). Follow-up over 2 years revealed no tumour recurrence.

Endocrine mucin-producing sweat gland carcinoma and associated primary cutaneous mucinous carcinoma: Review of the literature.

BACKGROUND: Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare, low-grade, cutaneous adnexal carcinoma with neuroendocrine differentiation. It is considered to be a precursor of invasive neuroendocrine type primary cutaneous mucinous carcinoma (PCMC). OBJECTIVE: To review clinicopathological literature summary of EMPSGC and associated neuroendocrine PCMC from all reported cases and compare its behavior vs non-neuroendocrine PCMC data reported in the literature. METHODS: A review of English literature of all EMPSGC cases with and without associated PCMC was carried out. RESULTS: EMPSGC was associated with invasive neuroendocrine type PCMC in 35.7% of cases. We found the recurrence rate of PCMC associated with EMPSGC of about 12.3%, which is much less than the 30% recurrence rate reported for the non-neuroendocrine subtype of PCMC. The non-neuroendocrine subtype of PCMC shows a 4% and 11% rate of distant and lymph node metastasis, respectively, vs EMPSGC-associated neuroendocrine type of PCMC, which is very indolent and only one recent case of locoregional metastasis out of 190 EMPSGC cases has been reported so far. LIMITATION: Limitation of our study includes data derivation from case reports and case series in the literature. CONCLUSION: The prognostic benefits for this cohort of patients may be considered in their overall management.

Early squamous cell carcinoma of the oral tongue with histologically benign lymph nodes: A model predicting local control and vetting of the eighth edition of the American Joint Committee on Cancer pathologic T stage.

BACKGROUND: The eighth edition of the American Joint Committee on Cancer staging manual (AJCC8) added depth of invasion to the definition of pathologic T stage (pT). In the current study, the authors assess pT stage migration and the prognostic performance of the updated pT stage and compare it with other clinicopathologic variables in patients with early squamous cell carcinoma of the oral tongue (OTSCC; tumors measuring ≤4 cm) with histologically benign lymph nodes (pN0). METHODS: A multi-institutional cohort of patients with early OTSCC was restaged as per AJCC8. Primary endpoints were local recurrence (LR) and locoregional recurrence (LRR). Influential variables were identified and an LR/LRR prediction model was developed. RESULTS: There were a total of 494 patients, with 49 LR and 73 LRR. AJCC8 pT criteria resulted in upstaging of 37.9% of patients (187 of 494 patients), including 34.5% (64 of 185 patients) from pT2 to pT3, without improving the prognostication for LR or LRR. Both LR and LRR were found to be similar for patients with AJCC8 pT2 and pT3 disease. On multivariate analysis, LR was only found to be associated with distance to the closest margin (hazard ratio, 0.36; 95% CI, 0.20-0.64 [P = .0007]) and perineural invasion (hazard ratio, 1.92; 95% CI, 1.10-0.64 [P = .046]). Based on these 2 predictors, a final proportional hazards regression model (which may be used similar to a nomogram) was developed. The proposed model appeared to be superior to AJCC pT stage for estimating the probability of LR and LRR for individual patients with early OTSCC. CONCLUSIONS: AJCC8 pT criteria resulted in pT upstaging of patients with pN0 disease without improved LR or LRR prognostication. The proposed model based on distance to the closest margin and perineural invasion, status outperformed pT as a predictor of LR and LRR in patients with early OTSCC.

Risk stratification of fine-needle aspiration cytology of parotid neoplasms based on the Milan system-Experience from a tertiary center in Asia.

BACKGROUND: The recently described Milan system provides a unified way of categorizing salivary gland fine-needle aspiration (FNA) cytology. We aim to use this system to stratify risk of malignancy in parotid FNAs. METHODS: In this retrospective case series, 376 FNAs were preoperatively performed for 573 parotidectomies over 14 years. RESULTS: Risk of malignancy on FNA is as follows: nondiagnostic 14.5%, non-neoplastic 26.7%, atypia of undetermined significance 29.3%, benign neoplasm 2.7%, neoplasm of uncertain malignant potential 19.1%, suspicious for malignancy 87.5%, and malignant 100%. The specific diagnoses of pleomorphic adenoma and Warthin tumor on FNA have high positive predictive value of 97.5% and 96.6%, respectively. Multivariate regression associates smaller size of lesion with a nondiagnostic or indeterminate result. Seniority of operator is associated with a lower likelihood of a nondiagnostic result. CONCLUSIONS: This large Asian series validates the Milan system as a valuable tool in stratifying malignancy risk of parotid FNAs.

Oral Neurothekeoma of the Right Buccal Mucosa.

Oral neurothekeoma or nerve sheath myxoma is a rare benign oral tumour of nerve sheath origin. Historically, this tumour has been subclassified as myxoid (classic), mixed, or the cellular type, depending on the amount of myxoid stroma and cellularity. We present a case of oral neurothekeoma (mixed type) of the buccal mucosa. The tumour was completely excised. No recurrence was detected in the last 3 years after local excision.