Pancreatic Paraganglioma: a Case Report and Literature Review

Paraganglioma is a rare tumor of paraganglia, derived from neural crest cells in sympathetic or parasympathetic ganglions. It can be widely distributed from the skull base to the bottom of the pelvis. The pancreas, however, is a rare location of this neoplasm, and only a limited number of cases have been reported in the English literature, especially with gadoxetic-acid-enhanced magnetic resonance imaging (MRI) and diffusion-weighted images (DWI). We herein report a case of pathologically proven paraganglioma in the pancreas head with a literature review on endoscopic ultrasonography (EUS), computed tomography (CT), gadoxetic-acid-enhanced MRI, and DWI sequence.

Pancreatic Paraganglioma: a Case Report and Literature Review

Paraganglioma is a rare tumor of paraganglia, derived from neural crest cells in sympathetic or parasympathetic ganglions. It can be widely distributed from the skull base to the bottom of the pelvis. The pancreas, however, is a rare location of this neoplasm, and only a limited number of cases have been reported in the English literature, especially with gadoxetic-acid-enhanced magnetic resonance imaging (MRI) and diffusion-weighted images (DWI). We herein report a case of pathologically proven paraganglioma in the pancreas head with a literature review on endoscopic ultrasonography (EUS), computed tomography (CT), gadoxetic-acid-enhanced MRI, and DWI sequence.

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.

Ultrasound features of secondary appendicitis in pediatric patients

PURPOSE: The purpose of this study was to evaluate the ultrasonographic findings of secondary appendicitis (SA) and to discuss the differential findings compared with primary appendicitis. METHODS: In this study, we analyzed the ultrasonographic findings of 94 patients under 15 years old of age treated at our institution from May 2005 to May 2014 who had bowel inflammation and an inflamed appendix with a maximal outer diameter >6 mm that improved with nonsurgical treatment (the SA group). Ninety-nine patients with pathologically proven acute appendicitis (the primary appendicitis [PA] group) from June 2013 to May 2014 and 44 patients with pathologically negative appendectomy results from May 2005 to May 2014 were also included to compare the ultrasonographic features of these conditions. A retrospective review of the ultrasonographic findings was performed by two radiologists. The clinical and laboratory findings were also reviewed. The results were statically analyzed using analysis of variance, the Pearson chi-square test, and the two-tailed Fisher exact test. RESULTS: Compared with PA, cases of SA had a smaller diameter (9.8 mm vs. 6.6 mm, P < 0.001), and were less likely to show periappendiceal fat inflammation (98% vs. 6%, P < 0.001) or an appendicolith (34% vs. 11%, P < 0.001). SA showed mural hyperemia on color Doppler ultrasonography as frequently as PA (P=0.887). CONCLUSION: The ultrasonographic features of SA included an increased diameter compared to a healthy appendix and the same level of hyperemia as in PA. However, the diameter was commonly in the equivocal range (mean diameter, 6.6 mm), and periappendiceal fat inflammation was rarely present in SA.

Acellular Dermal Matrix and Split-Thickness Skin Graft Stabilized by Negative Pressure Wound Therapy for Postburn Scar Contracture: A Case Report

Postburn scar contracture for ankle is commonly treated with contracture release procedure and skin graft but contracture recurrence rate is high. Contracture in grafted skin is inversely related to the graft thickness but full-thickness skin graft is limited in large defect coverage. Using ADM for ankle joint defect which was following ankle postburn scar contracture release procedure with grafting split-thickness skin may be an alternative reconstructive option. We present a 63-year-old man with postburn scar contracture and Marjolin ulcer on his left ankle. He originally had limited ankle movement function but surgical resection of skin malignancy and surrounding scar tissue released the ankle. Coverage using ADM and STSG was performed and NPWT was used for graft fixation. Ankle range of motion was satisfactory at postoperative follow-up. The ADM and STSG with NPWT could be considered an alternative reconstruction option after contracture release for ankle.

Differential diagnosis of primary cutaneous CD4+ small/medium T-cell lymphoproliferative lesions: A report of three cases

No abstract available.

Meningeal Solitary Fibrous Tumors with Delayed Extracranial Metastasis

BACKGROUND: The term solitary fibrous tumor (SFT) is preferred over meningeal hemangiopericytoma (HPC), because NAB2-STAT6 gene fusion has been observed in both intracranial and extracranial HPCs. HPCs are now considered cellular variants of SFTs. METHODS: This study analyzes 19 patients with STAT6-confirmed SFTs, who were followed for over 11 years in a single institution. Ten patients (10/19, 56.2%) had extracranial metastases (metastatic group), while the remainder (9/19) did not (non-metastatic group). These two groups were compared clinicopathologically. RESULTS: In the metastatic group, the primary metastatic sites were the lungs (n = 6), bone (n = 4), and liver (n = 3). There was a mean lag time of 14.2 years between the diagnosis of the initial meningeal tumor to that of systemic metastasis. The median age at initial tumor onset was 37.1 years in the metastatic group and 52.5 in the non-metastatic group. The 10-year survival rates of the metastatic- and non-metastatic groups were 100% and 33%, respectively. The significant prognostic factors for poor outcomes on univariate analysis included advanced age (≥45 years) and large initial tumor size (≥5 cm). In contrast, the patients with higher tumor grade, high mitotic rate (≥5/10 high-power fields), high Ki-67 index (≥5%), and the presence of necrosis or CD34 positivity showed tendency of poor prognosis but these parameters were not statistically significant poor prognostic markers. CONCLUSIONS: Among patients with SFTs, younger patients (<45 years) experienced longer survival times and paradoxically had more frequent extracranial metastases after long latent periods than did older patients. Therefore, young patients with SFTs require careful surveillance and follow-up for early detection of systemic metastases.

A Case of Eyelid Sebaceoma Mimicking Chalazion

PURPOSE: To report a rare case of sebaceoma misdiagnosed as chalazion. CASE SUMMARY: A 42-year-old female presented with a visible mass in her right lower eyelid. An elevated, hard mass was located at the margin of the right lower eyelid, and she had a history of incision and curettage under the clinical impression of chalazion. On eversion of the lower eyelid, the tarsal portion of the mass was visible as a white-yellowish lesion. The mass was excised under local anesthesia. A sebaceoma was diagnosed based on histopathological examinations. Immunohistochemical studies showed positive staining results for mutator L homologue 1 (MLH1), mutator S homologue 2 (MSH2), and mutator S homologue 6 (MSH6), and she had no past medical history or family history of internal malignancy, suggesting a low possibility of Muir-Torre syndrome. CONCLUSIONS: Eyelid sebaceoma should be considered as a differential diagnosis for refractory chalazion.

The Clinical Utility of FDG PET-CT in Evaluation of Bone Marrow Involvement by Lymphoma / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Bone marrow biopsy is a standard method for the evaluation of bone marrow infiltration by lymphoma; however, it is an invasive and painful procedure. Fluorodeoxyglucose positron emission tomography-computed tomography (FDG PET-CT) is a noninvasive imaging technique with the potential to detect bone marrow involvement by lymphoma. MATERIALS AND METHODS: We retrospectively reviewed medical records of lymphoma patients. All patients were examined by FDG PET-CT and iliac crest bone marrow biopsy for initial staging work-up. RESULTS: The study population comprised 94 patients (median age, 60 years; 56 males) with Hodgkin's lymphoma (n=8) or non-Hodgkin's lymphoma (n=86). Maximum standardized uptake values on the iliac crest of patients with lymphoma infiltrated bone marrow were significantly higher than those of patients with intact bone marrow (2.2+/-1.2 g/mL vs. 1.3+/-0.4 g/mL; p=0.001). The calculated values for FDG PET-CT during evaluation of bone marrow involvement were as follows: sensitivity 50%, specificity 96%, positive predictive value 80%, negative predictive value 85%, and positive likelihood ratio (LR+) 11.7. The value of LR+ was 16.0 in patients with aggressive subtypes of non-Hodgkin's lymphoma (NHL). CONCLUSION: FDG PET-CT could not replace bone marrow biopsy due to the low sensitivity of FDG PET-CT for detection of bone marrow infiltration in lymphoma patients. Conversely, FDG PET-CT had high specificity and LR+; therefore, it could be a useful tool for image-guided biopsy for lymphoma staging, especially for aggressive subtypes of NHL. In addition, unilateral bone marrow biopsy could be substituted for bilateral bone marrow biopsy in lymphoma patients with increased FDG uptake on any iliac crest.

Expression of Histone Deacetylases HDAC1, HDAC2, HDAC3, and HDAC6 in Invasive Ductal Carcinomas of the Breast / 한국유방암학회지

PURPOSE: DNA deacetylation by histone deacetylase (HDAC) is an important mechanism involved in the oncogenic tumorigenesis of breast cancer. Previous studies have reported an association of the estrogen receptor (ER) with HDACs and demonstrated the efficacy of HDAC inhibitors for the treatment of breast cancers via in vitro experiments. In this study, we examined the association of HDAC expression with clinicopathological parameters and disease-specific survival. METHODS: Immunohistochemical (IHC) analysis of HDAC1, HDAC2, HDAC3, and HDAC6 was performed using tissue microarrays in 300 invasive ductal carcinomas. IHC scoring was determined by multiplication of the intensity (0 to 3) and the proportion (0 to 4) of staining, and we classified tumors into low- and high-HDAC expression groups. RESULTS: High expression of HDAC1 was correlated with the molecular subtype (p=0.001) and human epidermal growth factor 2 (HER2) amplification (p=0.012). High expression of HDAC6 was correlated with a younger age (p<0.001), ER expression (p=0.025), progesterone receptor expression (p=0.034), molecular subtype (p=0.023), and HER2 amplification (p=0.011). High HDAC1 expression was correlated with luminal A tumors (p=0.001), while high HDAC6 expression was more common in luminal B tumors (p=0.023). Although the expression of HDACs did not exhibit prognostic significance in the entire cohort, high expression of HDAC1 and HDAC6 was associated with improved overall survival (OS) in patients with ER-positive tumors (p=0.017 and p=0.029, respectively), and high expression of HDAC2 was correlated with improved OS in ER-negative tumors (p=0.048) on univariate analysis. Furthermore, high HDAC6 expression was associated with improved disease-free survival (p=0.048) on multivariate analysis. CONCLUSION: HDAC1 expression is significantly correlated with the molecular subtypes of tumors, with the highest expression being observed in luminal A tumors. HDAC6 is a significantly correlated with ER expression and the molecular subtype, thereby supporting the estrogen regulatory property of HDAC6. HDAC1 and HDAC6 expression are good prognostic factors for ER-positive tumors.

Carcinoma Showing Thymus-like Differentiation (CASTLE) with Non-Recurrent Laryngeal Nerve: A Case Report / 대한갑상선학회지

Carcinoma showing thymus-like element (CASTLE) is a very rare malignant neoplasm in the lower portion of the thyroid gland or soft tissue of the neck. Recurrent laryngeal nerve (RLN) is the most frequent site of CASTLE. Non-RLN is also a rare anomaly. Both CASTLE and non-RLN are risk factors for vocal cord paralysis. In this report, the authors describe a 73-year-old patients diagnosed with CASTLE and non-RLN. During total thyroidectomy, one RLN was sacrificed inevitably because of tumor invasion, while the other non-RLN was successfully saved, which was expected based on preoperative computed tomography (CT). If the diagnosis is uncertain, CT should be checked to prevent unexpected risks.

Characteristics of Cutaneous Lymphomas in Korea According to the New WHO-EORTC Classification: Report of a Nationwide Study

BACKGROUND: Previously, cutaneous lymphomas were classified according to either the European Organization for the Research and Treatment of Cancer (EORTC) or the World Health Organization (WHO) classification paradigms. The aim of this study was to determine the relative frequency of Korean cutaneous lymphoma according to the new WHO-EORTC classification system. METHODS: A total of 517 patients were recruited during a recent 5 year-period (2006-2010) from 21 institutes and classified according to the WHO-EORTC criteria. RESULTS: The patients included 298 males and 219 females, and the mean age at diagnosis was 49 years. The lesions preferentially affected the trunk area (40.2%). The most frequent subtypes in order of decreasing prevalence were mycosis fungoides (22.2%), peripheral T-cell lymphoma (17.2%), CD30+ T-cell lymphoproliferative disorder (13.7%), and extranodal natural killer/T (NK/T) cell lymphoma, nasal type (12.0%). Diffuse large B-cell lymphoma accounted for 11.2% of cases, half of which were secondary cutaneous involvement; other types of B-cell lymphoma accounted for less than 1% of cases. CONCLUSIONS: In comparison with data from Western countries, this study revealed relatively lower rates of mycosis fungoides and B-cell lymphoma in Korean patients, as well as higher rates of subcutaneous panniculitis-like T-cell lymphoma and NK/T cell lymphoma.

Simultaneous Occurrence of Ductal Carcinoma In Situ within Juvenile Fibroadenoma in Both Breasts: A Brief Case Report

No abstract available.

A Case of Etoposide Therapy in a Patient Newly Diagnosed with Classic Kaposi's Sarcoma and Rheumatoid Arthritis / 대한내과학회지

Kaposi's sarcoma typically occurs in immunocompromised patients, especially those with acquired immunodeficiency syndrome. Human herpesvirus 8 (HHV-8) and human immunodeficiency virus (HIV) may play important roles in the development of Kaposi's sarcoma. We report the case of a 75-year-old male who presented with progressive multiple purplish papules and nodules on the skin for 3 months. Social and past medical histories seemed incompatible with an immunesuppressed condition and tests for HIV antibodies and HHV-8 were negative. He was newly diagnosed with rheumatoid arthritis, according to the 2010 ACR-EULAR criteria. Typical findings of Kaposi's sarcoma were confirmed by biopsy. After treatment with metronomic oral etoposide 25 mg once daily for 9 months, the skin lesions had almost resolved with no serious complication. Thus, we report a rare case of Kaposi's sarcoma developing in a patient with newly diagnosed rheumatoid arthritis who showed a good response to oral etoposide.

A Case of External Jugular Vein Thrombosis Originating from Phlebectasia / 대한이비인후과학회지

External jugular vein thrombosis is an infrequent disease that has been associated with altered blood flow, vascular endothelium lesion or a hypercoagulable state. It could be originated from phlebectasia, but very rare. It could be diagnosed with detailed history taking, when patients had no specific previous history of intervention or treatment. The authors report a case of external jugular vein thrombosis originated from phlebectasia without risk factor. This patient was successfully managed by ligation and excision of the vein without any complications.

Heterotopic Intestinal Cyst of the Submandibular Gland: A Case Study

Heterotopic gastrointestinal cysts are rarely found in the oral cavity. Most of these cysts are lined with gastric mucosa and involve the tongue. There have been no reported heterotopic intestinal cysts of the submandibular gland that are completely lined with colonic mucosa. An 8-year-old girl presented with an enlarging swelling in the left submandibular area, and a 4-cm unilocular cyst was fully excised. The cyst was completely lined with colonic mucosa that was surrounded by smooth muscle layer, and the lining cells were positive for CDX-2, an intestinal marker, indicating a high degree of differentiation. The pathogenesis remains unclear, but it may be related to the misplacement of embryonic rests within the oral cavity during early fetal development. Although heterotopic intestinal cysts rarely occur in the submandibular gland, they should be considered in the differential diagnosis of facial swellings in the pediatric population.

Hyaline Vascular Castleman Disease Involving Renal Parenchyma and a Lymph Node: A Case Report

Castleman disease is a rare lymphoproliferative lesion that is predominantly found in the mediastinum. Retroperitoneal and pararenal localizations are very rare. We describe a 36-year-old man with a hyaline vascular type of Castleman disease involving renal parenchyma and a paraaortic lymph node. Most reported renal Castleman disease was plasma cell type with systemic symptoms. Herein, we report the first Korean case of the hyaline vascular type of Castleman disease involving the renal parenchyma and the paraaortic lymph node simultaneously.

Expression of HAT1 and HDAC1, 2, 3 in Diffuse Large B-Cell Lymphomas, Peripheral T-Cell Lymphomas, and NK/T-Cell Lymphomas

BACKGROUND: It has generally been proven that histone acetylation and deacetylation are involved in the malignant transformation. To date, however, this has rarely been studied in cases of malignant lymphoma. METHODS: We studied nine cases of reactive lymphoid hyperplasia, 78 cases of diffuse large B-cell lymphoma (DLBCL), 13 cases of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), and 13 cases of extranodal NK/T-cell lymphoma, nasal type (NKTCL). Thus, we attempted to elucidate the associations of the degree of the expression of histone acetyltransferase 1 (HAT1), histone deacetylase (HDAC) 1, HDAC2, and HDAC3 with the clinical behaviors of above malignant lymphomas using the immunohistochemistry and a western blot analysis. RESULTS: The degree of the expression of HAT1 was higher in cases of DLBCL, PTCL-NOS or NKTCL as compared with reactive lymphoid hyperplasia (p0.05). CONCLUSIONS: HAT1, HDAC1, and HDAC2 play a critical role in the development of malignant lymphomas. Both HAT1 and HDAC1 might be indicators for a poor prognosis in cases of DLBCL as cooperating factors.

Fatal Cutaneous gamma/delta T-Cell Lymphoma with Central Nerve System Metastasis

Cutaneous gamma/delta T-cell lymphoma is an extremely rare and highly aggressive tumor that is often resistant to treatment, such as polychemotherapy and radiotherapy. Due to the complexity of clinical, pathologic, and immunohistochemical features of this disease entity, the physician should perform a careful evaluation; however, treatment should be rapid and aggressive. We present a case of fatal cutaneous gamma/delta T-cell lymphoma of a 55-year old woman who died after recurrence with central nerve system metastasis.

A Case of Gastric Lymphoepithelioma-like Carcinoma with Lymph Node Metastasis

Gastric lymphoepithelioma-like carcinoma is a very rare cancer of the stomach. It has undifferentiated malignant cells with rich lymphoid stroma histologically and its prognosis has known to be relatively fair. Recently, there were some reports on the successful removal of the lesion by endoscopic submucosal dissection technique. We present a case of 70 years old man who had 2 cm sized early gastric cancer of lymphoepithelioma-like carcinoma with perigastric lymph node metastasis, and also review the relevant medical literature.