Juan C. Hernandez-Prera

Changing Epidemiology of Hepatocellular Adenoma in the United States: Review of the Literature

Hepatocellular adenoma (HCA) is a benign neoplasm arising from hepatocytes. There is evidence that the inflammatory subtype may be associated with obesity and alcohol use and that men with metabolic syndrome may be at risk for malignant transformation of HCA. We sought to explore the combined experience of US centers as reported in the literature to document the epidemiologic shift in risk factors for HCA formation in the United States, namely, a shift from oral contraceptive pills (OCPs) to an emerging role of obesity as a contributing factor. Methods. Publications reporting HCA in the United States were identified through a PubMed search and a review of the literature. We excluded publications prior to 1970, single case reports, and publications for which there was no data available regarding patient characteristics including OCP use and the number of adenomas. Conclusion. Whereas earlier reports of HCA in the United States described cases exclusively in women exposed to OCPs, there is a trend towards an increase in HCAs reported in men, HCAs in the absence of OCP use, and increased reporting of multiple HCAs. This may be a result of newer OCP formulations and increasing prevalence of obesity.

Focal Nodular Hyperplasia and Hepatocellular Adenoma around the World Viewed through the Scope of the Immunopathological Classification

Focal nodular hyperplasia (FNH) and hepatocellular adenoma (HCA) are benign hepatocellular tumors. The risk of bleeding and malignant transformation of HCA are strong arguments to differentiate HCA from FNH. Despite great progress that has been made in the differential radiological diagnosis of the 2 types of nodules, liver biopsy is sometimes necessary to separate the 2 entities. Identification of HCA subtypes using immunohistochemical techniques, namely, HNF1A-inactivated HCA (35–40%), inflammatory HCA (IHCA), and beta-catenin-mutated inflammatory HCA (b-IHCA) (50–55%), beta-catenin-activated HCA (5–10%), and unclassified HCA (10%) has greatly improved the diagnostic accuracy of benign hepatocellular nodules. If HCA malignant transformation occurs in all HCA subgroups, the risk is by far the highest in the β-catenin-mutated subgroups (b-HCA, b-IHCA). In the coming decade the management of HCA will be more dependent on the identification of HCA subtypes, particularly for smaller nodules (<5 cm) in terms of imaging, follow-up, and resection.

Coexistence of inflammatory hepatocellular adenomas with HNF1α‐inactivated adenomas: is there an association?

To report the coexistence of inflammatory hepatocellular adenoma (IHCA) and HNF1α‐inactivated HCA (H‐HCA) in cases from a multicentre study.

The Ethical Implications of the Reclassification of Noninvasive Follicular Variant Papillary Thyroid Carcinoma

BACKGROUND Several studies have highlighted the lack of consensus in the diagnosis of follicular variant of papillary thyroid carcinoma (FVPTC). An international multidisciplinary panel to address the controversy was assembled at the annual meeting of the Endocrine Pathology Society in March of 2015, leading to the recent publication reclassifying encapsulated (or noninvasive) FVPTC (EFVPTC) as a benign neoplasm. Does this change in histologic taxonomy warrant a change in clinical practice, and how should it affect those who have been given this diagnosis in the past? We consider the financial and psychological impact of this reclassification and discuss the ethical, legal, and practical issues involved with sharing this information with the patients who are affected. SUMMARY The total direct and indirect cost of thyroid cancer surveillance in patients is significant. High levels of clinically relevant distress affect up to 43% of patients with papillary thyroid carcinoma, as estimated by the Distress Thermometer developed by the National Comprehensive Cancer Network for detecting distress in cancer patients. Although there are currently no legal opinions that establish a precedent for recontacting patients whose clinical status is altered by a change in nomenclature, the prudent course would be to attend to the requirements of medical ethics. CONCLUSION Informing patients with a previous diagnosis of EFVPTC that the disease has been reclassified as benign is expected to have a dramatic effect on their surveillance needs and to alleviate the psychological impact of living with a diagnosis of cancer. It is important to re-evaluate the pathologic slides of those patients at risk to ensure that the invasive nature of the tumor is comprehensively evaluated before notifying a patient of a change in diagnosis. The availability of the entire tumor for evaluation of the capsule may prove to be a challenge for a portion of the population at risk. We believe that it is the clinicians professional duty to make a sincere and reasonable effort to convey the information to the affected patients. We also believe that the cost savings with respect to the need for additional surgery, radioactive iodine, and rigorous surveillance associated with a misinterpretation of the biology of the diagnosis of EFVPTC in less experienced hands will likely more than offset the cost incurred in histologic review and patient notification.

Anisakidosis of the sigmoid colon disguising as metastatic carcinoma: A case report and review of the literature

Intestinal anisakidosis is a rare nematode infestation caused by the ingestion of larvae-infected raw or undercooked fish. Most cases are incidentally diagnosed during surgery for other reasons. We present such a case of anisakid larvae seen in a patients sigmoid colon resected for adenocarcinoma, where a subserosal nodule caused by the inflammatory reaction to the worm was presumed to be a lymph node involved by metastatic tumor. With the increasing popularity of raw fish consumption, the incidence of this parasitic infection is bound to increase, requiring surgical pathologists to be cognizant of its existence and aware of its histologic appearance.

Colitis cystica profunda indefinite for dysplasia in Crohn disease: A potential diagnostic pitfall

Colitis cystica profunda (CCP) is a nonneoplastic condition characterized by misplaced glands deep to the muscularis mucosae of the colon and may be difficult to differentiate from well-differentiated mucinous adenocarcinoma. Absence of dysplasia in CCP usually aids in this distinction. We present a challenging case of CCP in the setting of Crohn disease (CD) containing foci of atypical epithelium. A right hemicolectomy from a 46-year-old woman contained a stricture associated with a proximal multilocular cystic lesion containing mucin-filled glands dissecting through the colonic wall. These glands had lobulated architecture with smooth contours surrounded by lamina propria and lacking desmoplastic stroma. The epithelium had focal nuclear crowding, enlargement, and hyperchromasia, with increased nucleus to cytoplasm ratio, but overall preserved polarity. Atypical cells were focally positive for CK7 and p53, with increased MIB-1 staining. These findings were interpreted as indefinite for dysplasia. Chronic transmural inflammation and mucosal regeneration probably facilitated epithelial misplacement, which secondarily developed cytologic atypia. However, the overall architecture and lack of dysplasia in the overlying mucosa argue against a diagnosis of adenocarcinoma. Our case illustrates the difficult diagnosis of this uncommon but problematic phenomenon, awareness of which is paramount for pathologists and clinicians participating in the management of CD patients.

Intermittent facial spasms as the presenting sign of a recurrent pleomorphic adenoma.

The intimate anatomical relationship of the facial nerve to the parotid parenchyma has a significant influence on the presenting signs and symptoms, diagnosis and treatment of parotid neoplasms. However, to our knowledge, hyperactivity of this nerve, presenting as facial spasm, has never been described as the presenting sign or symptom of a parotid malignancy. We report a case of carcinoma arising in a recurrent pleomorphic adenoma of the left parotid gland (i.e., carcinoma ex pleomorphic adenoma) that presented with hemifacial spasms. We outline the differential diagnosis of hemifacial spasm as well as a proposed pathophysiology. Facial paralysis, lymph node enlargement, skin involvement, and pain have all been associated with parotid malignancies. To date the development of facial spasm has not been reported with parotid malignancies. The most common etiologies for hemifacial spasm are vascular compression of the ipsilateral facial nerve at the cerebellopontine angle (termed primary or idiopathic) (62%), hereditary (2%), secondary to Bell’s palsy or facial nerve injury (17%), and hemifacial spasm mimickers (psychogenic, tics, dystonia, myoclonus, myokymia, myorthythmia, and hemimasticatory spasm) (17%). Hemifacial spasm has not been reported in association with a malignant parotid tumor but must be considered in the differential diagnosis of this presenting symptom.

Pediatric Myofibroma of the Palate with Ulceration and Bone Destruction

Myofibroma is a rare benign neoplasm occurring in the head and neck, arising primarily in infants and children. Frequently, myofibromas grow rapidly leading to suspicion of malignancy and the potential for overaggressive surgical excision. We aim to report a rare case of myofibroma with ulceration and bone destruction. A nine-year-old female presented with an ulcerated left hard palate mass. Open biopsy was performed with pathology suggestive of myofibroma. A left partial maxillectomy and reconstruction with a buccal advancement flap were performed. Final pathology confirmed the diagnosis of a benign myofibroma. Myofibroma is a rare benign tumor of the head and neck which must be considered in the differential diagnosis by the clinician and the pathologist in order to prevent inappropriate and/or overaggressive treatment.

Enhanced interdisciplinary communication: development of an interactive thyroid nodule/cancer disease map: Enhanced Interdisciplinary Communication

Deficits related to inadequate clinical communication can result in incorrect diagnoses, inappropriate surgery, incorrect disease stratification, pathologic reporting, and/or interpretation. There are currently no validated or defined solutions to disease‐specific communication with regard to thyroid care.

Evaluation of Patients with Sinonasal and Ventral Skull Base Malignancies

A wide variety of tumors present in the sinonasal and ventral skull base. Patients often have nonspecific symptoms initially and present with advanced tumors, affecting the orbit and other adjacent structures. Evaluation of these malignancies with modern imaging techniques can define tumor invasion, but biopsy is often required to establish a diagnosis because most have a nonspecific appearance. A thorough understanding of the anatomy is the key to treatment planning, and a multidisciplinary approach determines the optimal strategy.