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1.  Castleman’s disease of the spleen 
Castleman’s disease (CD) is a rare lymphoproliferative disorder of unknown etiology. Clinically, it occurs as a localized (unicentric) disease or as a systemic (multicentric) disease. Unicentric Castleman’s disease (UCD) presents as a solitary mass and primarily affects the mediastinal, retroperitoneal, and cervical lymph nodes. In contrast to multicentric CD, which involves peripheral lymphadenopathy and numerous systemic symptoms, UCD is not typically associated with generalized symptoms. Three main distinct histologic variants are recognized: hyaline-vascular type, plasma cell type, and mixed type. Extranodal CD is rare. Specifically, UCD exclusively in the spleen is extremely rare, with only 2 cases described in the literature to date. Here, we describe an asymptomatic 75-year-old man with a 5.7 cm × 4.5 cm sized heterogenous enhanced mass located in the spleen. He underwent surgical resection for diagnosis and treatment. A pathologic examination indicated the hyaline-vascular type of CD. In this patient, the preoperative diagnosis was difficult to determine, and therefore, invasive procedures were required.
PMCID: PMC4316113  PMID: 25663790
Castleman’s disease; Hyaline-vascular type; Spleen
2.  Paraneoplastic secondary hypertension due to a renin-secreting desmoplastic small round cell tumor: A case report 
Oncology Letters  2014;8(5):1986-1992.
Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy with a poor outcome that occurs in adolescents and young adults; <200 cases of DSRCT have been reported. Renin-producing tumors are also rare and cases of extrarenal renin-producing tumors are even rarer. The present study describes the case of a 20-year-old male that was diagnosed with DSRCT and presented with severe hypertension and hypokalemia, as well as metabolic alkalosis. The plasma renin activity (PRA) level was identified to be markedly elevated (normal range in standing and supine positions, 1.3–4.0 ng/ml/h and 0.15–2.33 ng/ml/h, respectively) and the plasma aldosterone level was also increased (normal range in standing and supine positions, 4.0–31.0 ng/dl and 1.0–1.6 ng/dl, respectively). The symptoms of the patient were consistent with the renin-secreting tumor triad, which comprises hypertension, hypokalemia and elevated PRA. Paraneoplastic syndromes must always be considered in cancer patients exhibiting unusual clinical findings, despite their rarity. The current patient was diagnosed with paraneoplastic secondary hypertension due to the presence of disseminated renin-secreting DSRCT. The patient was treated with the VAC/IE regimen (vincristine, adriamycin, cyclophosphamide, ifosfamide and etoposide) for six cycles. Following this treatment, the serum renin and aldosterone levels fell to within the normal range and the patient’s blood pressure was normalized without antihypertensive medication. Although an immunohistochemical evaluation of renin was not conducted as the sample size was inadequate, the present study demonstrated that the tumor had produced renin. The biosynthesis of renin was identified by the presence of mRNA that coded for the renin precursor, which was observed in the ascites of the patient. The current study describes, to the best of our knowledge, the first reported case of paraneoplastic secondary hypertension in a patient presenting with a renin-producing DSRCT.
PMCID: PMC4186556  PMID: 25289084
desmoplastic small round cell tumor; aldosterone; plasma renin activity; paraneoplastic secondary hypertension; renin-producing tumor
3.  Evaluation of risk factors in patients with vitamin K-dependent coagulopathy presumed to be caused by exposure to brodifacoum 
Recently, many cases of vitamin K-dependent coagulopathy of unknown origin have been reported. Such patients lack any relevant family history and have no systemic disease, raising suspicion of superwarfarin intoxication. We evaluated individual risk factors causing coagulopathy and hemorrhagic symptoms in patients with suspected superwarfarin intoxication. In addition, we determined how to effectively treat vitamin K-dependent coagulopathy caused by suspected superwarfarin intoxication.
Seven patients with suspected superwarfarin intoxication who lacked any definitive history of rodenticide ingestion were included. Thirty-one patients initially diagnosed with rodenticide poisoning were also included. We performed a retrospective chart review of all subjects and examined clinical data including patient demographics and medical histories.
Patients initially diagnosed with rodenticide poisoning were divided into two groups, one of which had a laboratory abnormality (prothrombin time [PT] > 13 seconds) and another group with PTs in the normal range. There was no significant difference between the two groups in any of age, gender, the extent of chronic alcohol consumption, the causative rodenticide, psychiatric problems, ingestion of drugs interacting with warfarin, the extent of intoxication, or the type of ingestion attempt. The albumin level of the former group was significantly lower than that of the latter group (p = 0.014). Furthermore, a significant difference between the two groups was evident in terms of simultaneous ingestion of rodenticide and alcohol (p = 0.023).
Most patients with superwarfarin poisoning did not exhibit any complication. When such complications were evident, they were associated with serum albumin level and coingestion of rodenticide and alcohol.
PMCID: PMC4101597  PMID: 25045298
Vitamin K deficiency bleeding; Brodifacoum; Poisoning
4.  Bowel perforation associated sunitinib therapy for recurred gastric gastrointestinal stromal tumor 
Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. Several recent findings that there are activating mutations in the KIT and PDGFRA (platelet-derived growth factor receptor-α) genes of GISTs provide the rationale for using targeted therapies such as imatinib or sunitinib. Sunitinib, an oral multitargeted receptor tyrosine kinase inhibitor that inhibits kinases such as KIT, PDGFR (platelet-derived growth factor recepter), and VEGFR (vascular endothelial growth factor receptor), was recently approved for the treatment of imatinib-refractory GIST. Sunitinib is generally well tolerated and has an acceptable toxicity profile; an adverse event such as bowel perforation is rare. We present a patient with imatinib-refractory GIST who was successfully treated using sunitinib, but developed bowel perforation. The mechanism involved in bowel perforation associated with sunitinib is unknown. However, we presume that in our patient, the dramatic reduction in disseminated peritoneal metastases and bowel invasion of recurrent GIST during sunitinib treatment might have resulted in the bowel perforation.
PMCID: PMC3996717  PMID: 24783183
Gastrointestinal stromal tumors; Sunitinib; Intestinal perforation
5.  Comparison of laboratory characteristics between acute promyelocytic leukemia and other subtypes of acute myeloid leukemia with disseminated intravascular coagulation 
Blood research  2013;48(4):250-253.
Acute promyelocytic leukemia (APL) is an acute myeloid leukemia (AML) subtype with distinctive cell morphology, molecular presentation, clinical course, and treatment. About 90% of APL patients present with hemorrhagic complications due to disseminated intravascular coagulation (DIC). When APL is suspected, all-trans retinoic acid (ATRA) treatment is recommended even before confirmation by molecular tests. Specific criteria for differentiating unconfirmed APL from other AML subtypes with DIC are currently lacking. We aimed to achieve the early diagnosis of APL from other AML types with DIC by restricting the DIC criteria.
We retrospectively analyzed 29 patients newly diagnosed with AML accompanied by DIC from January 2005 to January 2013.
Fibrin degradation products (FDP) (77.7 µg/mL vs. 23.7 µg/mL, p=0.026), D-dimer (7,376.2 ng/mL vs. 1,315.2 ng/mL, p=0.018), and TIBC (264.4 µg/dL vs. 206.8 µg/dL, P=0.046) were higher, while fibrinogen (133.8 mg/dL vs. 373.2 mg/dL, p<0.001), WBC (14.988×109/L vs. 70.755×109/L, p=0.015), and ESR (7.1 mm/h vs. 50.0 mm/h, p <0.001) were lower in APL patients than in the patients with other AML subtypes. FDP ≥27 µg/mL, D-dimer ≥2,071 ng/mL, and fibrinogen ≤279 mg/dL were our threshold values. These markers may be characteristic to APL and helpful in presumptive diagnosis.
APL may be differentiated from other AML subtypes by core markers of DIC (FDP, D-dimer, and fibrinogen). We suggest that clinicians set new diagnostic thresholds by restricting the DIC criteria. These findings support the early initiation of ATRA, prior to confirmation by PML-RARA molecular testing.
PMCID: PMC3894382  PMID: 24466548
Acute myeloid leukemia; Acute promyelocytic leukemia; Disseminated intravascular coagulation
6.  Giant sized epidermal inclusion cyst of the breast initially mimicking a large fibroadenoma or phyllodes tumor 
Epidermal inclusion cysts are formed by inclusion of keratinizing squamous epithelium within the dermis, resulting in a cyst filled with lamellated keratin. These benign cysts are usually very small and intradermal subcutaneous lesions. They can occur anywhere in the body although they are more common on the face, trunk, neck, extremities and scalp. Only a few cases of epidermal cysts of the breast have been reported in the literature. An epidermal inclusion cyst of the breast can result in several problems, even if the size is unusual. We encountered a case of a giant sized epidermal inclusion cyst of the breast initially mimicking a large fibroadenoma or phyllodes tumor.
PMCID: PMC3412182  PMID: 22880186
Epidermal cyst; Breast neoplasms
7.  Giant anal condyloma (giant condyloma acuminatum of anus) after allogeneic bone marrow transplantation associated with human papillomavirus: a case report 
Condyloma acuminatum are caused by human papillomavirus. Giant condyloma acuminatum is a locally invasive, destructive, and large sized mass. Risk factors for the development of giant condyloma acuminatum include an immunodeficient state, such as human immunodeficiency virus infection, post-organ transplantation, or post-allogeneic bone marrow transplantation. However, reports of giant condyloma after bone marrow transplantation are extremely rare (0.3 to 1.3%). The standard treatment for giant condyloma acuminatum is recommended as wide surgical resection due to its high rate of success and low rate of recurrence.
Case presentation
A 31-year-old Korean man presented to our hospital with anal discomfort for more than one month due to a protruding mass. He had a history of BCR-ABL-positive acute lymphoblastic leukemia and had undergone an allogenic stem cell transplantation. Gross findings revealed a large perianal cauliflower-like mass over 7cm in size with invasion of the anal orifice. He was diagnosed with giant anal condyloma occurring after an allogeneic bone marrow transplantation. However, we achieved successful treatment using a combination of topical podophyllin and cryotherapy and transanal surgical excision, followed by bleomycin irrigation.
We report an extremely rare case of giant condyloma acuminatum of anus due to human papillomavirus type six in a patient with acute lymphoblastic leukemia following an allogeneic bone marrow transplantation. The tumor was successfully treated with a combination of topical podophyllin and cryotherapy and transanal surgical excision, followed by bleomycin irrigation.
PMCID: PMC4334849  PMID: 25597932
Condyloma; HPV; Allogenic bone marrow transplantation; Anus condyloma
8.  Colon cancer progression is driven by APEX1-mediated upregulation of Jagged 
The Journal of Clinical Investigation  2013;123(8):3211-3230.
Aberrant expression of apurinic-apyrimidinic endonuclease–1 (APEX1) has been reported in numerous human solid tumors and is positively correlated with cancer progression; however, the role of APEX1 in tumor progression is poorly defined. Here, we show that APEX1 contributes to aggressive colon cancer behavior and functions as an upstream activator in the Jagged1/Notch signaling pathway. APEX1 overexpression or knockdown in human colon cancer cell lines induced profound changes in malignant properties such as cell proliferation, anchorage-independent growth, migration, invasion, and angiogenesis in vitro and in tumor formation and metastasis in mouse xenograft models. These oncogenic effects of APEX1 were mediated by the upregulation of Jagged1, a major Notch ligand. Furthermore, APEX1 expression was associated with Jagged1 in various colon cancer cell lines and in tissues from colon cancer patients. This finding identifies APEX1 as a positive regulator of Jagged1/Notch activity and suggests that it is a potential therapeutic target in colon cancers that exhibit high levels of Jagged1/Notch signaling.
PMCID: PMC3726152  PMID: 23863623
9.  Solitary Lymphoblastic Lymphoma of the Thoracic Spine 
Non-Hodgkin's lymphoma rarely originates from bone, and even more infrequently from a vertebral body. Lymphoblastic lymphoma is a rare type of non-Hodgkin's lymphoma, and results from an abnormality in adaptive immune cells. A 27-year-old man presented with a two-month history of night sweats, weight loss, and severe back pain. Radiological studies demonstrated an osteolytic lesion compressing the subarachnoid space at the T11 level. Posterolateral fusion with decompression was performed and a pathologic examination confirmed lymphoblastic lymphoma of the B-cell precursor type. To our knowledge, this is the first report of solitary lymphoblastic lymphoma from B-cell precursors in of the thoracic spine. Herein, we discuss the presenting symptoms and the management of this rare case of lymphoblastic lymphoma.
PMCID: PMC3550428  PMID: 23346332
Lymphoblastic lymphoma; Spine
10.  Cutaneous Extraskeletal Osteosarcoma on the Scar of a Previous Bone Graft 
Annals of Dermatology  2011;23(Suppl 2):S160-S164.
Extraskeletal osteosarcoma (ESOS) is a very rare malignant tumor of mesenchymal origin. It is rarer than osseous osteosarcoma and there are very few reports of the skin being a primary site. Most reported cutaneous ESOS were accompanied with metastasis in other organs. A 56-year-old man presented with a painful, 1.5×0.8 cm sized, brown-colored nodule on the right girdle area for 3 months. The histologic findings revealed a tumor that was confined to the dermis without connection to the subcutaneous tissue. In addition, there were large amounts of thin and lace-like bony trabeculae and osteoid with neoplastic cells in a highly pleomorphic sarcomatous stroma.
PMCID: PMC3229055  PMID: 22148040
Cutaneous; Extraskeletal; Osteosarcoma; Scar

Results 1-10 (10)