Myxofibrosarcomas (MFSs), which consist of multiple nodules with a variable cellular population in a myxoid matrix, are primarily located in subcutaneous tissue. Pleomorphic hyalinizing angiectatic tumors (PHATs) are rare soft-tissue tumors characterized by a proliferation of highly pleomorphic spindle or polygonal cells and abundant ectatic blood vessels in cellular or myxoid stroma. We present here an unusual case of a high-grade MFS with a PHAT-like appearance. A 67-year-old man presented with an asymptomatic subcutaneous mass in the right forearm. The tumor had myxoid, hypo-, and hypercellular areas with highly pleomorphic spindle or polygonal tumor cells that showed frequent mitoses and nuclear pseudoinclusions. Foci of punctuate necrosis and inflammatory infiltration were present throughout the tumor, and abundant ectatic, thick-walled vessels containing blood clots were noted. The tumor cells were immunohistochemically positive for vimentin but negative for CD34, S-100 protein, smooth muscle actin, desmin, and bcl-2.
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Children's kinetic family drawings and their internalizing problem behaviors Jin Kyung Kim, Joo Hyun Suh The Arts in Psychotherapy.2013; 40(2): 206. CrossRef
Odontogenic keratocysts are benign intraosseous tumors of odontogenic origin that occur most commonly in the jaw. In particular, they have a predilection for the angle and ascending ramus of the mandible. In contrast, odontogenic keratocysts arising in the maxillary sinus are relatively rare. Two such cases are reported herein. In addition, the English literature that concerns odontogenic keratocysts of the maxillary sinus is reviewed.
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Evaluation of the prognostic role of SOX2 as a tumor stem cell marker in odontogenic cysts and tumors: clinical, radiographic and immunohistochemical correlation Haneen Mahmoud Zeen El-Abdeen, Mohamed Abdel-Monem Tawfik, Sherif Yousef Elnagdy, Noha Ahmed Mansour BMC Oral Health.2025;[Epub] CrossRef
Diagnostic Dilemma of Odontogenic keratocyst Mimicking a Dentigerous Cyst: A Case Report Georgia Benitha, Syed Wali Peeran International Journal of Head and Neck Pathology.2023; 6(2): 6. CrossRef
Keratocystic odontogenic tumor associated with impacted maxillary third molar extending to the antrum: A challenging diagnosis Roozbeh Pahlevan, Farnaz Keyhanlou, Sahar Fazel, Fatemeh Shahsavari Human Pathology: Case Reports.2019; 15: 59. CrossRef
Current Concepts and Occurrence of Epithelial Odontogenic Tumors: II. Calcifying Epithelial Odontogenic Tumor Versus Ghost Cell Odontogenic Tumors Derived from Calcifying Odontogenic Cyst Suk Keun Lee, Yeon Sook Kim Korean Journal of Pathology.2014; 48(3): 175. CrossRef
Keratocystic Odontogenic Tumor with an Ectopic Tooth in Maxilla Basavaraj T. Bhagawati, Manish Gupta, Gaurav Narang, Sharanamma Bhagawati Case Reports in Dentistry.2013; 2013: 1. CrossRef
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Keratocystic odontogenic tumour (KCOT) misdiagnosed as a dentigerous cyst Seema Chaudhary, Ashish Sinha, Pranamee Barua, Rachappa Mallikarjuna BMJ Case Reports.2013; 2013: bcr2012008741. CrossRef
We report two cases of ectopic epididymal ducts and efferent ductules in the testicular appendices (TAs) of adult men with normally descended testes. In both cases, a sessile TA was incidentally found at the upper pole of the right testis during the scrotal hydrocelectomy. Microscopically, a few closely arranged tubules were detected within the TA. In the first case, the tubules were lined with a pseudostratified columnar epithelium with numerous, long microvilli, and were surrounded by a smooth muscle coat. In contrast, in the second case, the tubules had a wavy luminal surface, because ciliated columnar cells alternated with groups of cuboidal cells. In both cases, strong CD10 immunoreactivity was observed in the luminal border of the lining epithelium.
Surgical pathologists should be aware of the presence of both ectopic epididymal ducts and efferent ductules that can occur in TAs, in order to avoid misinterpretation as transected, functional reproductive structures.
Wolf-Hirschhorn syndrome (WHS) is a malformation associated with a hemizygous deletion of the distal short arm of chromosome 4. Herein we report a fetal autopsy case of WHS.
A male fetus was therapeutically aborted at 17(+0) weeks gestational age, due to complex anomaly and intrauterine growth retardation, which were found in prenatal ultrasonography. His birth weight was 65 g. Mild craniofacial dysmorphism, club feet, bilateral renal hypoplasia, edematous neck, and left diaphragmatic hernia of Bochdalek were found on gross examination. On GTG-banding, the fetus revealed 46,XY,add(4p) karyotype and the mother revealed 46,XX,t(4;18)(p16;q21.1), with normal karyotype of the father. Array comparative genomic hybridization performed on the autopsied lung tissue revealed loss of 4p16.2-->4pter and gain of 18q21.1-->18qter, suggesting 46,XY,der(4)t(4;18)(p16.2;q21.1)mat of fetal karyotype. This suggested deletion of 4p, compatible with WHS inherited from the mal-segregation of a maternal translocation t(4;18)(p16.2;21.1). Therefore, our fetus was both genotypically and phenotypically compatible with WHS.
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A Case of Wolf-Hirschhorn Syndrome with Periventricular Nodular Heterotopia Presenting with Status Epilepticus Bong Sul Suh, Keun Hyeok Ko, Kon Hee Lee, Tae-Jung Sung Neonatal Medicine.2015; 22(4): 233. CrossRef
Rhabdoid glioblastoma is distinguishable from classical glioblastoma by cytogenetics and molecular genetics Sun-Ju Byeon, Hwa Jin Cho, Hae Woon Baek, Chul-Kee Park, Seung-Hong Choi, Se-Hoon Kim, Hee Kyung Kim, Sung-Hye Park Human Pathology.2014; 45(3): 611. CrossRef
Myoepitheliomas are well-established to occur in the salivary glands, but they have also been described in the breast, upper aerodigestive tract, skin, and soft tissues.
We report here on a unique case of primary myoepithelioma that occurred in the right testis of a 28-year-old man. The tumor was entirely confined to the testis and it was clearly separated from the epididymis. Histopathology revealed mixed architectural patterns in which the reticular areas merged into the chondromyxoid stroma. The tumor cells, which were focally immunoreactive to pancytokeratin and S-100 protein, were round to ovoid and spindly arranged in cords, strands, and fascicles. They showed mild nuclear pleomorphism, sparse mitotic figures and a low Ki-67 proliferative index. There was no ductal differentiation in the tumor. To the best of our knowledge, there has been only one case report of a primary testicular myoepithelioma in the English medical literature.
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Primary cutaneous myoepithelial carcinoma: a case report and review of the literature Markus Winther Frost, Torben Steiniche, Tine Engberg Damsgaard, Lars Bjørn Stolle APMIS.2014; 122(5): 369. CrossRef
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Cystic nephroma (CN) is a benign cystic neoplasm composed of mixed epithelial and stromal elements. Less than 200 cases have been reported. We had a patient, a 41-year-old woman, who had a huge typical CN. The patient was admitted for a right renal mass that was found incidentally. On laparaoscopic right nephrectomy, there was an encapsulated 7 cm multilocular cystic mass at the upper pole.
Microscopically, the cystic wall was lined by a single layer of low cuboidal or hobnail epithelium without a solid area.
The thin septa were composed of bland, ovarian type spindle cells. The main differential diagnoses were mixed epithelial and stromal tumor (MEST), low grade multilocular renal cell carcinoma, and tubulocystic carcinoma. The results of immunohistochemical staining were cytokeratin 7/19(+/+) and CD10(-) in lining epithelium, estrogen receptor/progesterone receptor(+/+) in stromal cells. After surgery, she was free of recurrence for 10 months. We report this rare case and compare it with other cystic renal tumors, especially MEST.
Serous cystadenomas of the pancreas account for approximately a third of pancreatic cystic neoplasms. Their coexistence with a second tumor is extremely rare. We now report a case of a serous microcystic adenoma combined with an intraductal papillary mucinous tumor of the pancreas in a 69-year-old man. Abdominal computed tomography scans demonstrated an incidental cystic mass in the body with cystic dilatation of the duct in the head of the pancreas.
Central pancreatectomy with pancreatico-jejunostomy, and cyst excision of the pancreatic head were performed.
Histologic examination demonstrated a serous microcystic cystadenoma in the body coexisting with an intraductal papillary mucinous adenoma in the head of the pancreas. This case study highlights the importance of careful intra-operative and pathologic examination for synchronous pancreatic tumors.
Diffuse pulmonary meningotheliomatosis (DPM) is an extremely rare condition. We herein report a unique case of DPM in a 54-year-old woman with a previous history of hepatocellular carcinoma. A chest computed tomography showed diffuse bilateral nodular infiltration, suggesting miliary spread of metastatic hepatocellular carcinoma. The patient underwent a video-assisted thoracoscopic surgery for diagnostic purposes. The cut surface of the lung specimen showed multiple dispersed small nodules, consisting of variably sized nests or whorls of bland epithelioid cells often along the walls of alveolar septa or in a perivascular network within the alveolar interstitium. The tumor cells showed immunoreactivity for epithelial membrane antigen, vimentin, and progesterone receptor. DPM should be included in the differential diagnosis of diffuse multiple small nodules or a reticular pattern in the radiologic studies.
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Minute Pulmonary Meningothelial-Like Nodules Simulating Hematogenous Lung Metastasis: A Case Report Sang Kook Lee, Gi Jeong Kim, Young Jae Kim, Ah Young Leem, Eu Dong Hwang, Se Kyu Kim, Joon Chang, Young Ae Kang, Song Yee Kim Tuberculosis and Respiratory Diseases.2013; 75(2): 67. CrossRef
Chronic sclerosing sialadenitis, Mikulicz disease or Kuttner tumor has been recently recognized as a spectrum of IgG4-related sclerosing disease. IgG4-related disease is characterized by a high serum IgG4 level and tissue infiltration of IgG4-positive plasmacytes. We report three cases of chronic sclerosing sialadenitis with variably associated systemic involvement. All patients presented with a submandibular mass or swelling, and all the resected submandibular glands showed diffuse lymphocytic infiltration, lymphoid follicles, and septal fibrosis. Two of the specimens revealed numerous IgG-positive plasma cells, most of which were IgG4-positive on immunohistochemical staining. One of them was associated with dacryoadenitis and hypophysitis. The other patient had ureterorenal lesions. Immunohistochemical study was unavailable in remaining one case, but the histologic features along with elevated IgG level and associated pancreatitis supported the diagnosis. All patients received steroid therapy postoperatively and are doing well. Salivary gland involvement in IgG4-related fibrosclerosis should be recognized in systemic medical pathology.
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A Case of Immunoglobulin G4-Related Sialadenitis and Dacryoadenitis Kyoung Kyu Lee, Hun Po Cho, Young Min Lee, Jun Ho Park Korean Journal of Otorhinolaryngology-Head and Neck Surgery.2013; 56(7): 444. CrossRef
We herein describe a case of a 55-year-old healthy woman with localized primary thymic amyloidosis presented as a mediastinal mass, found incidentally by chest radiography.
Computed tomography revealed a 4.1 cm soft tissue lesion with nodular calcification in the left anterior mediastinum.
The resected specimen was a well-defined lobulating mass with calcification. Microscopically, the mass was consisted of amorphous eosinophilc hyalinized substances involving the thymus and intrathymic lymph nodes. These eosinophilic substances showed apple-green bi-refringence under polarized light after staining with Congo red. In immunohistochemical study, they were positive for kappa and lambda light chains and negative for amyloid A. There was no evidence of systemic amyloidosis in clinical investigations. A final diagnosis of localized primary thymic amyloidosis was made.
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Benign ductal or glandular neoplasms of the esophagus unrelated to Barrett esophagus are extremely rare. Only 9 cases have been reported in the English language literature.
We now report a case of esophageal gland duct adenoma incidentally found in a 73-year-old man. A 0.8 cm-sized, polypoid submucosal lesion in the distal esophagus was removed. Histologically, the lesion was well circumscribed and consisted of several ducts or cysts with focal papillary configurations. Interstitial lymphocytic infiltration with germinal centers was also observed. The lining cells of ducts or cysts were composed of two layers: an inner intensely eosinophilic luminal duct cell layer and an outer myoepithelial cell layer that was accentuated by alpha-smooth muscle actin. There was no significant nuclear atypia or mitosis. Mucin production was occasionally observed in a few goblet cells. To the best of our knowledge, this is the first case of benign ductal or glandular neoplasm of the esophagus among Koreans.
The incidence of severe hepatitis A virus (HAV) infection has been increasing. However, clinicopathologic features of severe HAV infection that lead to liver transplantation (LT) have not been reported in Korea. We retrieved 16 LT cases with HAV infection during the last 3 years at Asan Medical Center, Seoul, Korea. Fifteen cases progressed to hepatic encephalopathy. Thirteen cases survived with or without complications, and three patients died of sepsis. The explanted liver showed massive or zonal necrosis with moderate to severe cholestasis. The zonal distribution of necrosis was frequently associated with endothelialitis of portal and/or central veins. Degenerative changes of hepatocytes were various in degree and distribution. Viral inclusions were suspected in two cases. Although HAV infection is usually confirmed by serological tests, significant venulitis of central and/or portal veins and viral inclusions, which are rarely observed, can suggest an HAV infection as a cause of massive hepatic necrosis of unknown mechanism.
In situ follicular lymphoma is a newly defined entity among the lymphoid neoplasms and is defined as architecturally normal-appearing lymph nodes and other lymphoid tissues that have one or more follicles that demonstrate bcl-2 overexpressing centrocytes and centroblasts, with or without a monomorphic cytologic appearance suggestive of follicular lymphoma. Here we present a case of in situ follicular lymphoma diagnosed during the follow-up after a complete response to the treatment of lymphocyte-rich classical Hodgkin's lymphoma. In our case, because only a few germinal centers contained bcl-2 overexpressing cells, we missed the diagnosis of in situ follicular lymphoma in the initial histological examination. We could establish the diagnosis only after performing bcl-2 immunostaining in the sequential biopsy. Therefore, we recommend that careful histological examination along with bcl-2 immunostaining is needed in patients with suspicious clinical findings.
We report the first case of an angiomyomatous hamartoma (AH) of the popliteal lymph nodes (LNs) occurring in association with diffuse pigmented villonodular synovitis (PVNS) of the knee. AH is a rare benign vascular disease with a predisposition for the LNs of the inguinal region.
Twenty-five cases of AH have been reported to date; however, the precise pathogenesis is still undetermined. In the present case, an open synovectomy revealed two of three popliteal LNs in close proximity to the extra-articular component of diffuse PVNS. These LNs demonstrated irregularly distributed thick-walled blood vessels in the hilum. These vessels extended into the medulla and cortex and were associated with haphazardly arranged smooth muscle cells in the sclerotic stroma. These findings are compatible with an AH. Our observations raise the possibility that AH of the popliteal LNs may represent an abnormal proliferative reaction against the inflammatory process caused by PVNS of the knee.
Bilateral frontal polymicrogyria is a recently recognized syndrome characterized by symmetric polymicrogyria of both frontal lobes that presents with delayed motor and language development, spastic quadriparesis, and variable mental retardation. However, the postmortem findings of this syndrome are not fully elaborated. Here we describe an autopsy case of bilateral frontal polymicrogyria in a male fetus delivered at 22 weeks gestation due to extensive chorioamnionitis. The microscopic findings included a thinned cortical plate with fair neuronal maturation. There were no signs of neuronal damage and the white matter was unremarkable.
Brucellosis is a zoonosis caused by several species of Brucella. Brucellosis is usually an acute or sub-acute febrile illness that histologically develops granulomatous inflammation. Brucella prostatitis is a very rare complication and is usually accompanied by epididymo-orchitis. We now report a case of histologically proven granulomatous prostatitis due to Brucella without clinical evidence of epididymo-orchitis. A 61-year-old farmer presented with myalgia, low back pain, and fever. A needle biopsy of the prostate was performed due to symptoms of urinary frequency and high prostate specific antigen levels (17.3 ng/mL). Histologically, the prostate showed granulomatous inflammation without caseous necrosis.
Polymerase chain reaction (PCR) studies of blood and prostatic tissue for Brucella were positive, while a PCR study for Mycobacterium tuberculosis was negative. The patient was treated with doxycycline and rifampin. A possibility of Brucella prostatitis should be considered in the differential diagnosis of granulomatous prostatitis or prostatitis of unknown origin associated with or without epididymo-orchitis.
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The patient was a 52-year-old female with swelling in both lower legs and peripheral blood eosinophilia. Biopsy specimen revealed the heavy infiltration of eosinophils with sparse small lymphocytes showing mild atypia. The diagnosis was Kimura disease. The symptoms including eosinophilia were relieved by steroid treatment. At 17 months from initial biopsy, the patient developed swelling of the buttock. At 25 months, fever and dyspnea with multiple lung nodules developed. Wedge resection revealed multiple aggregates of CD3(+), CD56(+), Epstein-Barr virus(+) large atypical lymphocytes with necrosis. The patient was finally diagnosed with extranodal NK/T cell lymphoma (NKTL). Epstein-Barr virus in situ hybridization retrospectively performed on the previous biopsies demonstrated Epstein-Barr virus infection in small CD3(+) lymphocytes. The patient expired after 26 months despite chemotherapy. Blood eosinophilia correlated well with disease activity during the clinical course. This case shows not only unusual histologic features, which hampered the correct diagnosis, but also a unique clinical manifestation of NKTL.
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Here we report a case of Strongyloides stercoralis infection of the gastric and pancolonic mucosa in a 79-year-old female with a monoclonal gammopathy of undetermined significance.
Endoscopic biopsies were performed in gastric antrum, cecum, distal ascending colon, and hepatic flexure of the colon. On microscopic examination, there were many adult worms, larvae and eggs in the gastric and colonic mucosa. Worms, larvae, and eggs were located in the crypts and within the lumen of the crypts. The body wall of the adult worm was composed of cuticle and a weak muscle layer. A routine stool examination failed to detect larvae or ova. Based on the histopathologic examination, these parasites were confirmed as S.
stercoralis.
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Sclerosing polycystic adenosis (SPA) of the salivary glands is a rare entity analogous to fibrocystic disease of the breast. Less than 50 cases of SPA have been published in the literature. We report the first Korean case of SPA of the right parotid gland. A 34-year-old man presented with a slowly growing right parotid mass. Computed tomography showed a relatively well-demarcated, heterogeneously enhancing mass with multiple small calcifications. Fine needle aspiration showed cohesive sheets of epithelial cells with granular oncocytic cytoplasm and scattered lymphocytes.
The parotidectomy specimen showed a 3 cm-sized solid nodular lesion with small cysts. Microscopically, the lesion was an unencapsulated mass of sclerotic fibrous tissue with cystic ducts, multiple calcifications, and lymphoplasma cell infiltration. Sclerosing adenosis and cystic ducts with frequent apocrine-like cells were noted. Familiarity with the cytologic and histological features of SPA is very important making the correct diagnosis.
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The origin of osteoclast-like giant cell tumor (OGCT) of the salivary gland has been debated because the prototypic cells of osteoclast-like cells and mononuclear stromal cells are largely unexplained in this gland. Bone marrow-derived CD14+ and CD45+ monocyte-derived multipotential cells (CD14+/CD45+ MOMC) may be one of the possible origins of OGCTs of salivary glands, which have never been explored in salivary OGCTs. We present a case of OGCT accompanied with carcinoma ex pleomorphic adenoma in the parotid gland of a 67-year-old Korean female. The tumor presented as a rapidly growing cervical mass comprising a central area of carcinoma ex pleomorphic adenoma and a peripheral circumferential area of OGCT. The immunohistochemical staining pattern was phenotypically consistent with bone marrow-derived CD14+/CD45+ MOMC. This case is the first report of a salivary OGCT in Korea.
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Fibromatosis can occur at various sites, but intracranial fibromatosis is exceptionally rare. Here, we report a case of intracranial fibromatosis arising in the suprasellar area of a 52-year-old woman who had undergone a surgery at that site. A computed tomography scan revealed a heavily calcified, highly enhancing, poorly demarcated mass in the left sellar area that extended into the left suprasellar, parasellar areas, and orbital apex and completely encased the left distal inferior cerebral artery. Histologic and immunohistochemical features were compatible with those of fibromatosis, although the cellularity was focally higher than usual. The etiology of extra-abdominal fibromatosis is unknown, but physical injuries such as trauma and irradiation have been reported to be associated with its occurrence. Although fibromatosis is rare in the intracranial area, it should be considered as a differential diagnosis when an intracranial mass occurs at a previously injured site.
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Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor consisting of distinctive perivascular epithelioid cells, and is commonly detected in the uterus.
The liver is an uncommon site for primary PEComa. In this study, we report a case of primary hepatic PEComa in a 36-year-old woman. Upon gross examination, the tumor was a well-defined, brownish solid mass, measuring 6.5x5.2x4.5 cm.
Microscopically, the tumor consisted largely of epithelioid cells and some spindle cells with a clear to eosinophilic cytoplasm and a rich network of delicate capillaries in the stroma. With the exception of their relatively large size and microscopically sinusoidal infiltrative growth pattern, all other histopathologic features of the tumor were consistent with their being benign. The tumor cells were positive for human melanoma black-45 and smooth muscle actin, and negative for cytokeratin-cocktail and c-kit.
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A teratoma is a germ-cell tumor composed of tissue components representing derivatives of three germ layers. A teratoma in the region of adrenal gland is a rare retroperitoneal tumor. We now report a case of a primary adrenal teratoma. A 38-year-old woman presented with an incidentally detected adrenal mass. The computed tomography scan revealed a 9x8x7.5 cm fat density mass with calcification in the left adrenal gland. The surgically resected tumor was round and well circumscribed and the adrenal gland was present at the periphery of the tumor. The cut surface contained fat tissue and a hair containing cyst.
Microscopically, the tumor consisted of adipose tissue, hair, skin appendage, nerve, muscle bundle and bone.
We describe a 69-year-old woman who presented with a dedifferentiated extraskeletal myxoid chondrosarcoma arising in the left masticator space. Computed tomography and magnetic resonance imaging revealed a 5 cm sized mass in the left masticator space. Histologically, the tumor consisted of two distinct areas. The less cellular area was a low-grade extraskeletal myxoid chondrosarcoma, composed of strands or cords of uniform spindle cells and abundant myxoid stroma. The more cellular, dedifferentiated area corresponded to a high grade myxofibrosarcoma, consisting of anaplastic tumor cells in myxoid stroma and geographic necrosis. The tumor cells of the former area were positive for S-100 protein, microtubule-associated protein-2 (MAP-2) and class III beta-tubulin, but negative for cytokeratin, smooth muscle actin, and desmin. The tumor cells in the latter, pleomorphic area showed MAP-2 and beta-tubulin immunoreactivity with a high Ki-67 labeling index. Based on its histologic and immunohistochemical features, the tumor was considered a dedifferentiated extraskeletal myxoid chondrosarcoma.
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