A multicenter study of interobserver variability in pathologic diagnosis of papillary breast lesions on core needle biopsy with WHO classification.

BACKGROUND: Papillary breast lesions (PBLs) comprise diverse entities from benign and atypical lesions to malignant tumors. Although PBLs are characterized by a papillary growth pattern, it is challenging to achieve high diagnostic accuracy and reproducibility. Thus, we investigated the diagnostic reproducibility of PBLs in core needle biopsy (CNB) specimens with World Health Organization (WHO) classification. METHODS: Diagnostic reproducibility was assessed using interobserver variability (kappa value, κ) and agreement rate in the pathologic diagnosis of 60 PBL cases on CNB among 20 breast pathologists affiliated with 20 medical institutions in Korea. This analysis was performed using hematoxylin and eosin (H&E) staining and immunohistochemical (IHC) staining for cytokeratin 5 (CK5) and p63. The pathologic diagnosis of PBLs was based on WHO classification, which was used to establish simple classifications (4-tier, 3-tier, and 2-tier). RESULTS: On WHO classification, H&E staining exhibited 'fair agreement' (κ = 0.21) with a 47.0% agreement rate. Simple classifications presented improvement in interobserver variability and agreement rate. IHC staining increased the kappa value and agreement rate in all the classifications. Despite IHC staining, the encapsulated/solid papillary carcinoma (EPC/SPC) subgroup (κ = 0.16) exhibited lower agreement compared to the non-EPC/SPC subgroup (κ = 0.35) with WHO classification, which was similar to the results of any other classification systems. CONCLUSIONS: Although the use of IHC staining for CK5 and p63 increased the diagnostic agreement of PBLs in CNB specimens, WHO classification exhibited a higher discordance rate compared to any other classifications. Therefore, this result warrants further intensive consensus studies to improve the diagnostic reproducibility of PBLs with WHO classification.

Enterovesical Fistula From Meckel Diverticulum.

Meckel diverticulum is a common congenital malformation of the gastrointestinal tract and can cause complications such as ulceration, hemorrhage, intussusception, and perforation. This report describes a very rare complication of an enterovesical fistula associated with chronic Meckel diverticulum. A 51-year-old male presented with over 10 years of persistent pyuria. Tests were performed to rule out malignancy, including serum prostate-specific antigen level, urine cytology, bacterial culture, cystoscopy, and bladder computed tomography. An enterovesical fistula was identified, and laparoscopic exploration was performed. The findings suggested enterovesical fistula formation caused by chronic inflammation at the tip of a Meckel diverticulum. Segmental resection of the small bowel including the diverticulum and primary repair of the urinary bladder along with partial cystectomy were performed. The postoperative clinical course was uneventful. An enterovesical fistula is a very rare complication resulting from chronic inflammation of a Meckel diverticulum.

A Comparison of Results after Anterior Cruciate Ligament Reconstruction in over 40 and under 40 Years of Age: A Meta-Analysis.

PURPOSE: Anterior cruciate ligament (ACL) injury is one of the most common injuries that occur in the knee, and ACL reconstruction (ACLR) is commonly performed for preventing aggravation of degenerative changes and restoring of knee stability in young, athletic patients. This meta-analysis has a purpose of evaluating the clinical and arthrometrical outcomes of ACLR in a group of middle age patients (40 years and older) and comparing with patients under 40 years of age. METHODS: MEDLINE, EMBASE, the Cochrane Central Register of Controlled Trials, Web of Science, and SCOPUS electronic databases were searched for relevant articles comparing the outcomes of ACLR between younger and older than 40 years of age until December 2016. Data searching, extraction, analysis, and quality assessment were performed based on the Cochrane Collaboration guidelines. Clinical outcomes were evaluated and compared between groups. The results were presented as mean difference for continuous outcomes with 95% confidence intervals whereas risk ratio for binary outcomes. RESULTS: Seven studies were included in the meta-analysis. Based on International Knee Documentation Committee classification, side-to-side difference, Tegner activity score, Lysholm knee score, there were no significant clinical and mechanical differences between the groups. CONCLUSIONS: This meta-analysis confirmed that after ACLR, middle age (＞40 years) and young age (＜40 years) patients did not present with significant difference in clinical and arthrometric results.

Myoferlin Expression and Its Correlation with FIGO Histologic Grading in Early-Stage Endometrioid Carcinoma.

BACKGROUND: For endometrioid carcinoma patients, International Federation of Gynecologists and Obstetricians (FIGO) histologic grading is very important for identifying the appropriate treatment method. However, the interobserver discrepancy with this three-tiered grading system is a serious potential problem. In this study, we used immunohistochemistry to analyze the relationship between FIGO histologic grading score and myoferlin expression. METHODS: We studied the endometrioid carcinoma tissues of 60 patients from Gyeongsang National University Hospital between January 2002 and December 2009. Immunohistochemical analysis of myoferlin was performed on tissue microarray blocks from surgical specimens. RESULTS: Myoferlin expression was observed in 58 of 60 patients. Moderate and strong myoferlin expression was observed in low-grade endometrioid carcinoma, while there was a tendency toward loss of myoferlin expression in high-grade endometrioid carcinoma (p<.001). CONCLUSIONS: Our study revealed that myoferlin loss is significantly correlated with high FIGO grade of endometrioid carcinoma.

Metaplastic spindle cell carcinoma of the breast in a patient with neurofibromatosis type 1.

We report a unique case of a 67-year-old woman with neurofibromatosis type 1, who was also diagnosed with metaplastic spindle cell carcinoma of the left breast. She had many neurofibromatosis lesions on her body, as well as the mass in the left breast. After the breast mass was diagnosed as a malignant mesenchymal tumor by core needle biopsy, the patient underwent left modified radical mastectomy. Subsequently, the pathological analysis of the tumor showed it to be a metaplastic spindle cell carcinoma. The co-occurrence of neurofibromatosis type 1 and breast cancer, in particular metaplastic spindle cell carcinoma, is very rare.

Left sinus of valsalva aneurysm ruptured into left ventricle: A case report of 320-multidetector CT findings.

RATIONALE: Ruptured aneurysm originating from the left coronary sinus of Valsalva into the left ventricle (LV) is extremely rare. Imaging features of sinus aneurysm has been commonly reported using echocardiography or angiography. Here, we report multidetector computed tomography (MDCT) findings of left sinus of Valsalva aneurysm extending into the LV and caused severe aortic regurgitation (AR) in a 44-year-old male with latent infective endocarditis. The role of MDCT in preoperative surgical planning was also emphasized. PATIENT CONCERNS: The patient visited our hospital due to worsening exertional dyspnea for 3 months. DIAGNOSES: On cardiac computed tomography (CT) using 320-MDCT, a saccular aneurysm arising from the left coronary sinus of Valsalva extending into the LV was diagnosed as the cause of severe AR. INTERVENTIONS: The patient underwent resection of the aneurysm, aortic root reconstruction, and aortic valve replacement. OUTCOMES: The patient made an uneventful recovery. Follow-up echocardiography showed no paravalvular leakage with improved LV function. LESSONS: MDCT with wide coverage and high temporal resolution can provide exact and comprehensive information about complicated conditions, leading to confident surgical planning and successful management.

Thymoma and Synchronous Primary Mediastinal Seminomas with Florid Follicular Lymphoid Hyperplasia in the Anterior Mediastinum: A Case Report and Review of the Literature.

Thymoma is the most common neoplasm of the anterior mediastinum and has malignant potential. Germ cell tumors (GCTs) found in the anterior mediastinum are usually benign, and malignant GCTs, such as seminomas, are rare. Histologically, mediastinal seminoma is indistinguishable from testicular seminoma except for site-associated morphological features such as lymphoid follicular hyperplasia. Therefore, excluding metastasis is very important. Recently, we treated a young adult patient with multiple thymic masses that occurred simultaneously. The patient underwent a thymectomy for the removal of the mediastinal masses, one of which was diagnosed as type B2 invasive thymoma, and two of which were diagnosed as primary mediastinal seminomas with massive follicular hyperplasia. The patient received adjuvant chemotherapy after surgical resection. To our knowledge, this is the first description of a thymoma and a mediastinal seminoma occurring simultaneously in the thymus. We present this case along with a literature review.

A Case of Severe HIV-Associated Psoriasis Successfully Treated with Acitretin Therapy.

Psoriasis is a chronic inflammatory skin disease that involves immune-mediated cutaneous inflammation and keratinocyte hyperproliferation. Psoriasis in patients with HIV responds poorly to treatment and has a high morbidity rate, thus posing a challenge to clinicians. Until now, there have been no documented cases of acitretin therapy for HIV-associated psoriasis in Korea. Here, we report a case of safe and successful therapy with acitretin in a 52-year-old man with HIV-associated psoriasis that responded poorly to previous treatments including steroids and ultraviolet B phototherapy. We also review the relevant literature.

Urachal cyst presenting with huge abscess formation in adults.

Urachal disease, a disorder where embryonic remnant of the cloaca and the allantois present after birth as a midline fibrous cord, is usually detected in infancy and childhood. But urachal disease in adults is rare. We report a case of a huge abscess derived from a urachal cyst in an adult. A 52-year-old man presented with peri-umbilical distension and abdominal pain for 2 weeks. Ultrasonography and abdominal computed tomography scan demonstrated a huge abscess derived from the abdominal wall. After prompt incision and drainage, the remaining abscess cavity was removed completely under general anesthesia. Pathologic report was consistent with urachal duct cyst, and the patient was discharged in a week without complication.

Is stromal microinvasion in primary mucinous ovarian tumors with "mucin granuloma" true invasion?

Stromal microinvasion has been recently described in ovarian mucinous borderline tumors (MBTs), leading to proposals for new classifications, including a category of MBTs with stromal microinvasion. This study was conducted to test the validity of this belief. To determine whether stromal microinvasion can be distinguishable from extruded neoplastic epithelium from an adjacent ruptured gland, particularly if accompanied by a mucin granuloma (MG) on hematoxylin and eosin (H&E)-stained sections, we compared the histopathologic features of 138 primary ovarian mucinous tumors, consisting of 81 MBTs, 37 MBTs with stromal microinvasion, 11 intraglandular carcinomas, 2 with microinvasive foci, and 7 mucinous adenocarcinomas with extensive stromal invasion. Immunohistochemical analysis for cytokeratin was performed in 72 cases. Of 77 cases containing MGs, 52% contained "microinvasive foci," consisting of individually scattered epithelial or irregular glandular components in the intervening stroma, as determined by H&E staining. Of the 37 negative cases on H&E, cytokeratin reactivity was detected in 15 additional cases with epithelial/glandular components. Therefore, "stromal microinvasion" can be missed on H&E. All patients with MBT with or without microinvasive foci and localized intraglandular carcinoma with or without microinvasive foci had stage I tumors; none died of tumor-related causes during the follow-up period. Tumor-related deaths were identified only in 5 patients with extensive stromal invasion. This study suggests that some cases of stromal microinvasion in stage I MBTs not accompanied by high grade nuclear atypia represent mucocele-like stromal reaction to ruptured mucinous glands rather than true stromal invasion because isolated tumor cells were exclusively confined to the boundary of MGs and devoid of obvious cellular atypia, and no patient with stromal microinvasion died of tumor-related causes.

SMARCB1/INI1 missense mutation in mucinous carcinoma with rhabdoid features.

Malignant rhabdoid tumor (MRT) is a rare and aggressive tumor associated with deletion or mutation of a tumor suppressor gene SMARCB1/INI1, a member of the SWI/SNF chromatin-remodeling complex. Reported herein is a case of pancreatic mucinous carcinoma accompanying rhabdoid features with immunohistochemical and ultrastructural studies as well as analysis of the SMARCB1/INI1 gene. A 65-year-old woman presented with a 2 month history of abdominal and chest pain. A well-defined grayish tan fish-flesh mass (11 x 9 x 7 cm) with focal mucinous area was present in the pancreatic tail. Microscopically, the tumor had a biphasic growth pattern: a mucinous carcinoma component and a poorly differentiated carcinoma component with rhabdoid features showing loosely cohesive cells with abundant eosinophilic cytoplasm, displaced nuclei, and prominent nucleoli. The rhabdoid component coexpressed vimentin and cytokeratin. Sequencing analysis of the DNA extracted from the mucinous and rhabdoid components showed a missense mutation CCC to ACC in codon 116 of the SMARCB1/INI1 gene. Being aware of rhabdoid features would help diagnose this rare and aggressive malignant tumor and may provide an opportunity for further evaluation of SMARCB1/INI1 gene alteration and determination of its prognostic significance.

Proximal-type epithelioid sarcoma with elevated serum CA 125: report of a case with CA 125 immunoreactivity.

Proximal-type epithelioid sarcoma is a recently described rare soft tissue neoplasm. It is commonly found in the pelvic, perineal, and genital areas in middle-aged or older adults, as compared with the classic type of epithelioid sarcoma, which arises in the distal portion of the extremities in adolescents and young adults. Proximal-type epithelioid sarcoma has a more aggressive clinical behavior than the classic type of epithelioid sarcoma. Proximal-type epithelioid sarcoma is histologically characterized by a diffuse proliferation of epithelioid cells with prominent rhabdoid feature. Recently, a few cases have been reported of epithelioid sarcoma with elevated serum CA 125 level and CA 125 immunoreactivity in neoplastic cells. These cases raise the possibility that serum CA 125 and CA 125 immunoreactivity could be a useful tumor marker for diagnosing and monitoring epithelioid sarcoma. We describe a case of proximal-type epithelioid sarcoma with elevated serum CA 125 level (up to 3395 U/mL [reference range, <35 U/mL]) in a 12-year-old girl who presented with a huge pelvic mass measuring 12 cm in greatest dimension. The serum CA 125 level dropped to 452 U/mL after a debulking operation of the mass. Immunostaining for CA 125 demonstrated a positive immunoreactivity in the neoplastic cells. She received one cycle of chemotherapy and died of the disease 2 months after diagnosis. This case represented a rare example of proximal-type epithelioid sarcoma with elevated serum CA 125 and immunoreactivity for CA 125 in the tumor cells. Based on the previous reported cases and the current case, serum CA 125 as well as immunohistochemical stain for CA 125 may be a useful tumor marker of proximal-type epithelioid sarcoma.

Diagnostic and pathogenetic significance of increased stromal apoptosis and incomplete vasculogenesis in complete hydatidiform moles in very early pregnancy periods.

Hydropic swelling, trophoblastic proliferation, and stromal avascularity of chorionic villi are the key features of advanced cases of complete hydatidiform moles (CHMs). Recently, however, the use of high-resolution ultrasonography has enabled earlier detection of CHMs, and these show previously unrecognized histologic features such as numerous immature vascular networks, nonhydropic hypercellular stroma, and frequent karyorrhexis in the villous stroma. To determine whether stromal vasculogenesis is affected in CHMs of very early pregnancy period (VECM), we compared the number of mature and immature blood vessels and their precursors in the villous stroma and counted the rates of stromal apoptosis and proliferation, as defined by immunopositivity for cleaved caspase-3 and Ki-67, in 63 cases of VECM, 11 cases of partial hydatidiform mole (VEPM), and 10 samples of normal placental tissue (NP) before the 13th gestational week. Using antibody to CD31, we found that the number of mature blood vessels with distinct lumen was significantly reduced in both VECM and VEPM compared with NP (P<0.001), but the number of CD31-positive primitive stromal cells or immature vascular networks without lumen did not differ significantly among the three groups. Stromal apoptotic rate was significantly higher in VECM than in VEPM or NP (P<0.001), which was very useful in differential diagnosis. Our results suggest that complete vasculogenic differentiation is significantly retarded in VECM due to increased apoptosis in the precursor components of blood vessels. It may result in a lack of vascular drainage and cause progressive accumulation of vesicular fluid in the later gestational period.

Primary carcinoid tumor of the bilateral testis associated with carcinoid syndrome.

Carcinoid tumors derived from neuroendocrine cells can release serotonin and other vasoactive substances into the systemic circulation, resulting in carcinoid syndrome. Testicular carcinoid, a rare disease accounting for less than 1% of all testicular neoplasms, rarely manifests symptoms of carcinoid syndrome. We describe a case of carcinoid syndrome arising from a primary testicular carcinoid tumor. A 21-year-old male patient presented with facial flushing and diarrhea for 5 years. He had an enlarged left testis and a 1-cm, ill-defined, hard, non-tender mass in his right testis. His 24 h urinary excretion of 5-hydroxyindoleacetic acid was elevated (16.1 mg/day). Somatostatin receptor scintigraphy correlated with carcinoid tumor in both testes. Following bilateral orchiectomy, the patient's facial flushing and diarrhea disappeared.