Outcomes of Yttrium-90 Radioembolization for Unresectable Combined Biphenotypic Hepatocellular-Cholangiocarcinoma.

PURPOSE: To evaluate outcomes of yttrium-90 radioembolization in patients with combined biphenotypic hepatocellular-cholangiocarcinoma (cHCC-CC). MATERIALS AND METHODS: A retrospective review of patients with biopsy-confirmed cHCC-CC treated with yttrium-90 radioembolization between 2012 and 2018 was performed. Twenty-two patients with cHCC-CC (mean age 65.6 y, 17 men, 5 women) underwent 29 radioembolization treatments (5 resin, 24 glass microspheres). Survival data were available in 21 patients, and hepatic imaging response data were available in 20 patients. Hepatic imaging response to radioembolization was assessed on follow-up CT or MR imaging using modified Response Evaluation Criteria In Solid Tumours criteria. Univariate stepwise Cox regression analysis was used to evaluate the association between demographic and clinical factors and survival. Logistic regression evaluated associations between clinical factors and response to treatment, overall response, and disease control. RESULTS: Hepatic imaging response was as follows: 15% complete response, 40% partial response, 10% stable disease, and 35% progressive disease (55% response rate, 65% disease control rate). Two patients were downstaged or bridged to transplant, and 1 patient was downstaged to resection. Median overall survival was 9.3 mo (range, 2.5-31.0 mo) from time of radioembolization. Nonreponse to treatment, bilobar disease, presence of multiple tumors, and elevated carbohydrate antigen 19-9 before treatment were associated with reduced survival after radioembolization. CONCLUSIONS: Radioembolization is a viable option for locoregional control of cHCC-CC with good response and disease control rates.

Contrast-Enhanced Spectral Mammography is Comparable to MRI in the Assessment of Residual Breast Cancer Following Neoadjuvant Systemic Therapy.

PURPOSE: To evaluate the performance of contrast-enhanced spectral mammography (CESM) compared to MRI in the assessment of tumor response in breast cancer patients undergoing neoadjuvant systemic therapy (NST). METHODS: The institutional review board approved this study. From September 2014 to June 2017, we identified patients with pathologically confirmed invasive breast cancer who underwent NST. All patients had both CESM and MRI performed pre- and post-NST with pathological assessment after surgical management. Size of residual malignancy on post-NST CESM and MRI was compared with surgical pathology. Lin concordance and Pearson correlation coefficient were used to assess agreement. Bland-Altman plots were used to visualize the differences between tumor size on imaging and pathology. RESULTS: Sixty-five patients were identified. Mean age was 52.7 (range 30-76) years. Type of NST included chemotherapy in 53 (82%) and endocrine therapy in 12 (18%). Mean tumor size after NST was 14.6 (range 0-105) mm for CESM and 14.2 mm (range 0-75 mm) for MRI compared with 19.6 (range 0-100) mm on final surgical pathology. Equivalence tests demonstrated that mean tumor size measured by CESM (p = 0.009) or by MRI (p = 0.01) was equivalent to the mean tumor size measured by pathology within - 1 and 1-cm range. Comparing CESM versus MRI for assessment of complete response, the sensitivity was 95% versus 95%, specificity 66.7% versus 68.9%, positive predictive value 55.9% versus 57.6%, and negative predictive value 96.7% versus 96.9% respectively. CONCLUSIONS: CESM was comparable to MRI in assessing residual malignancy after completion of NST.

Response to Loco-Regional Therapy Predicts Outcomes After Liver Transplantation for Combined Hepatocellular-Cholangiocarcinoma.

INTRODUCTION AND AIM: Combined hepatocellular-cholangiocarcinoma (HCC-CCA) is a rare liver malignancy distinct from either hepatocellular carcinoma (HCC) or cholangiocarcinoma. Liver transplantation (LT) is not recommended for HCC-CCA because of suboptimal outcomes. Non-invasive diagnosis of HCC-CCA is extremely challenging; thus, some HCC-CCAs are presumed as HCC on imaging and listed for LT with the correct diagnosis ultimately made on explant pathology. We compared HCC-CCA with HCC to determine the utility of response to pre-transplant loco-regional therapy (LRT) in predicting outcomes for HCC-CCA after LT as a potential means of identifying appropriate HCC-CCA patients for LT. MATERIAL AND METHODS: Retrospective review of 19 patients with pathologically confirmed HCC-CCA were individually matched to 38 HCC patients (1:2) based on age, sex, and Milan criteria at listing was performed. The modified response evaluation criteria in solid tumors was used to categorize patients as responders or non-responders to pre-transplant LRT based on imaging performed before and after LRT. Overall survival (OS) and recurrence-free survival (RFS) were examined. RESULTS: OS at 3 years post-transplant was 74% for HCC-CCA and 87% for HCC. RFS at 3 years was 74% for HCC-CCA, and 87% for HCC. Among responders to LRT, the 3-year OS was 92% for HCC-CCA and 88% for HCC; among non-responders, 3-year OS was 43% for HCC-CCA and 83% for HCC. Higher 3-year OS was observed among HCC-CCA responders (77%) compared with HCC-CCA non-responders (23%). CONCLUSIONS: OS was similarly high among.

Liver transplantation in patients with incidental hepatocellular carcinoma/cholangiocarcinoma and intrahepatic cholangiocarcinoma: a single-center experience.

BACKGROUND: Reports of liver transplantation (LT) in patients with mixed hepatocellular carcinoma/cholangiocarcinoma (HCC/CC) and intrahepatic cholangiocarcinoma (ICC) are modest and have been mostly retrospective after pathological categorization in the setting of presumed HCC. Some studies suggest that patients undergoing LT with small and unifocal ICC or mixed HCC/CC can achieve about 40%-60% 5-year post-transplant survival. The study aimed to report our experience in patients undergoing LT with explant pathology revealing HCC/CC and ICC. METHODS: From a prospectively maintained database, we performed cohort analysis. We identified 13 patients who underwent LT with explant pathology revealing HCC/CC or ICC. RESULTS: The observed recurrence rate post-LT was 31% (4/13) and overall survival was 85%, 51%, and 51% at 1, 3 and 5 years, respectively. Disease-free survival was 68%, 51%, and 41% at 1, 3 and 5 years, respectively. In our cohort, four patients would have qualified for exception points based on updated HCC Organ Procurement and Transplantation Network imaging guidelines. CONCLUSIONS: Lesions which lack complete imaging characteristics of HCC may warrant pre-LT biopsy to fully elucidate their pathology. Identified patients with early HCC/CC or ICC may benefit from LT if unresectable. Additionally, incorporating adjunctive perioperative therapies such as in the case of patients undergoing LT with hilar cholangiocarcinoma may improve outcomes but this warrants further investigation.

Squamous Cell Carcinoma In Situ Overlying Merkel Cell Carcinoma.

BACKGROUND: Merkel cell carcinoma (MCC) is a rare and aggressive cutaneous neoplasm that has exhibited an exponential increase in incidence in the past 3 decades. Combined MCC and cutaneous squamous cell carcinoma (SCC/MCC) is an uncommon variant of MCC that exhibits worse prognosis than pure MCC. OBJECTIVE: To describe the clinical presentation, dermoscopy, and histology of an unusual subtype of combined SCC/MCC. METHODS AND RESULTS: A 73-year-old white woman presented with an ulcerated and violaceous 10-mm plaque on her right jawline that had been present for 2 to 3 months. On dermoscopy, the lesion was predominantly milky pink to red with peripheral crusting and large-caliber polymorphous vessels. Histology revealed SCC in situ above and adjacent to MCC. The tumor was excised with clear margins, and sentinel lymph node scintography was negative for nodal involvement.

Papillary glioneuronal tumor with a high proliferative component and minigemistocytes in a child.

Papillary glioneuronal tumor (PGNT) is a rare type of primary brain tumor. Although PGNT has traditionally been defined as a clinically indolent neoplasm, several cases with high proliferative activity and tumor recurrence have recently been reported. We report a case of PGNT in a 12-year-old boy who presented with epilepsy and harbored a 64 mm cystic tumor with a high proliferative component in the right temporal lobe. (11) C-methionine positron emission tomography (PET) showed high uptake in the solid mass. Gross total resection of the tumor mass was achieved and the patient became seizure-free without any neurological deficits. Histologically, the tumor contained two distinct areas of a vasocentric papilliform structure and a desmoplastic component. Minigemistocytic cells and small necrotic regions were observed adjacent to the pseudopapillae. Immunohistochemical analyses revealed both glial and neuronal differentiation. The Ki-67 proliferation index was high (14%) in the area corresponding to the high uptake region in the (11) C-methionine PET. No tumor recurrence was observed 20 months after surgery. High proliferative PGNTs are rare and to our knowledge this is only the third pediatric case of PGNT with atypical features reported in the literature. Hence, we here review the reported cases of PGNT and discuss the clinical, radiological and histological features of this malignancy.

Malignant mixed epithelial and stromal tumor of the kidney: report of the first male case.

Mixed epithelial and stromal tumor of the kidney is a rare benign tumor that consists of both epithelial and stromal cells. To date, eight malignant cases have previously been reported in female patients only. We report the first case of malignant mixed epithelial and stromal tumor of the kidney in a male patient. A 67-year-old Japanese man receiving hormonal therapy for prostatic cancer was found to have a right renal cystic tumor and underwent right nephrectomy. Histologically, the tumor was composed of benign epithelial and stromal cells in addition to malignant undifferentiated stromal cells. Immunohistochemically, the malignant stromal component was positive for cluster of differentiation 99 and B-cell lymphoma 2, but no chimeric transcripts for synovial sarcoma were identified. Finally, a diagnosis of malignant mixed epithelial and stromal tumor of the kidney was recorded. Urologists and pathologists should recognize that malignant mixed epithelial and stromal tumors of the kidney might occur in male patients receiving hormonal therapy for prostatic cancer.

Complex orbital angiomyoma with features of a lymphangiohemangioma.

A 31-year-old woman developed left frontal headache and retrobulbar pain with exotropia and mild limitation of upgaze without proptosis. Imaging studies disclosed a circumscribed mass measuring 1.5 cm in the greatest diameter. At surgery, the lesion was adherent to the surrounding normal orbital tissues, making for a difficult and dangerous resection. Histopathologically and immunohistochemically, the lesion was a mixed cavernous angiomatous (CD31-positive) and lymphangiomatous (D2-40-positive) tumor with abundant interstitial smooth muscle. Such lesions can present significant surgical challenges due to incomplete pseudoencapsulation.

Pure and Mixed Tubular Carcinoma of the Breast: Mammographic Features, Clinicopathological Characteristics and Prognostic Analysis.

Objective: To evaluate the mammographic features, clinicopathological characteristics, treatments, and prognosis of pure and mixed tubular carcinomas of the breast. Materials and methods: Twenty-five tubular carcinomas were pathologically confirmed at our hospital from January 2011 to May 2019. Twenty-one patients underwent preoperative mammography. A retrospective analysis of mammographic features, clinicopathological characteristics, treatment, and outcomes was performed. Results: Altogether, 95% of the pure tubular carcinomas (PTCs) and mixed tubular carcinomas (MTCs) showed the presence of a mass or structural distortions on mammography and the difference was not statistically significant (P = .373). MTCs exhibited a larger tumor size than PTCs (P = .033). Lymph node metastasis was more common (P = .005) in MTCs. Patients in our study showed high estrogen receptor and progesterone receptor positivity rates, but low human epidermal growth factor receptor 2 positivity rate. The overall survival rate was 100% in both PTC and MTC groups and the 5-year disease-free survival rates were 100% and 75%, respectively with no significant difference between the groups (P = .264). Conclusion: Tubular carcinoma of the breast is potentially malignant and has a favorable prognosis. Digital breast tomosynthesis may improve its detection. For patients with PTC, breast-conserving surgery and sentinel lymph node biopsy are recommended based on the low rate of lymph node metastasis and good prognosis. MTC has a relatively high rate of lymph node metastasis and a particular risk of metastasis. Axillary lymph node dissection should be performed for MTC even if the tumor is smaller than 2 cm.

Laparoscopic resection of a large mixed epithelial-stromal tumour of the seminal vesicle: a rare entity and review of the current literature.

Mixed epithelial-stromal tumours (MESTs) of the seminal vesicle (SV) are a rare neoplasm, with biological behaviour ranging from benign to malignant. Due to their rarity, there are no established guidelines for their treatment. We report a 37-year-old man with a large MEST of the SV which was successfully resected by laparoscopic transperitoneal approach. Amidst the controversy regarding the nomenclature and grading of MESTs in literature, we reclassified the previous reports of MESTs incorporating both the WHO and Reikie et al grading.

Digital dermoscopic changes during follow-up of de-novo and nevus-associated melanoma: a cohort study.

BACKGROUND: Nevus-associated melanoma (NAM) has been regarded as a distinct biological entity from de-novo melanoma (DNM); however, static dermoscopy often fails in differentiating these entities. Digital dermoscopic monitoring allows to identify dynamic changes occurring during follow-up; this may improve diagnostic accuracy and potentially our knowledge on NAM biology. We aimed to define main independent factors associated with NAM diagnosis and those influencing follow-up time in a population of melanomas excised at follow-up. METHODS: A cohort of melanomas excised at follow-up was retrospectively and consecutively selected. NAMs and DNMs were compared according to baseline features and main dermoscopic changes occurring during follow-up. Univariate and multivariable logistic and Cox's regression analysis were performed to respectively define factors associated with NAM diagnosis and those influencing the risk for excision. RESULTS: Eighty-six melanomas were enrolled, of which 21 (24.4%) were nevus-associated. During follow-up NAMs mainly underwent atypical network modifications (47.6%), followed by inverse network (28.6%) and dermoscopic island (23.8%) worsening or appearance. DNMs were also mainly characterized by atypical network modifications (47.7%), however, a significant proportion of cases underwent irregular pigmentation/dots/globules or regression changes (29.2%), which were rarely seen among NAMs. Furthermore, both multivariable logistic and Cox's regression analysis demonstrated a significant association between NAM and a longer follow-up. CONCLUSIONS: We demonstrated that among melanomas excised at follow-up, different patterns of dermoscopic changes may be found between NAMs and DNMs. This finding, together with the association of NAM with a longer follow-up time, supports the hypothesis of different biological behavior of these two entities.