Colloid carcinoma of the uterine cervix: a case report with respect to immunohistochemical analyses.

Colloid carcinoma, characterized by the presence of a large amount of extracellular mucin that results in the formation of mucous lakes with a relative paucity of neoplastic glandular cells within them, is extremely rare in the uterine cervix. Herein, we report an additional case of colloid carcinoma of the cervix and discuss the immunohistochemical characteristics and histogenesis of this extremely rare tumor. A 47-year-old Japanese female without any history of carcinomas was found to have a bulky mass in the cervix. Biopsy from the cervix revealed adenocarcinoma; subsequently, total hysterectomy was performed. Histopathologic study demonstrated that columnar or cuboidal neoplastic glandular cells forming cribriform or tubular structures floated within the mucous lakes involving almost the entire layer of the cervical wall. Adenocarcinoma in situ (AIS) component was also observed. Immunohistochemically, tumor cells of the colloid carcinoma were positive for cytokeratin 7, MUC5AC, MUC6, and p16 (diffuse), but negative for cytokeratin 20, MUC2, and cdx-2. In addition, human papillomavirus 16 was detected in both colloid carcinoma and AIS components. This is the first reported case of endocervical type colloid carcinoma, and the second documented case of cervical colloid carcinoma with immunohistochemical analyses of mucin. The present case had an endocervical type AIS component, which suggests that AIS may be a precursor lesion of colloid carcinoma. Moreover, this case clearly demonstrates that the occurrence of cervical colloid carcinoma correlates with high-risk human papillomavirus.

World wide web-based cytological analysis of atypical squamous cells cannot exclude high-grade intraepithelial lesions.

OBJECTIVE: It has been reported that the low level of consistency of diagnosis of atypical squamous cells cannot exclude high-grade squamous intraepithelial lesions (ASC-H) in uterine cervical cancer screening using the Bethesda System, indicating the necessity of a large-scale survey. We presented cases cytologically judged as ASC-H on our website and invited our members to give their opinions regarding the diagnosis by voting online. The Web voting results were analyzed and ASC-H was cytologically investigated. STUDY DESIGN: Virtual slides of atypical cells in cytology preparations of 53 cases were prepared and presented on a website. ASC-H cases were divided into 42 cases sampled by brush scraping and 11 cases sampled by cotton swab scraping. Fifty-three cases cytologically judged as ASC-H were classified into benign and CIN2/3, and their patterns of arrangement of atypical cells and 8 cytological parameters were morphologically investigated. RESULTS: The frequency of ASC-H diagnosis in the Web votes was low: 29.2% for brush-scraped and 26.2% for cotton swab-scraped cases. Three-dimensionality, coarse chromatin and irregular nuclei were significantly different between high-grade squamous intraepithelial lesions and benign cases. CONCLUSION: Web-based surveys showed the difference of cytological findings between high-grade squamous intraepithelial lesions and benign cases. To increase interobserver consistency, it may be useful to share information online, which avoids geographical and temporal limitations.

Cytological analysis of atypical squamous epithelial cells of undetermined significance using the world wide web.

OBJECTIVE: The low-level consistency of the cytodiagnosis of uterine cervical atypical squamous epithelial cells of undetermined significance (ASC-US) employing the Bethesda System has been reported, suggesting the necessity of a wide survey. We presented cases judged as ASC-US on the Web and analyzed the voting results to investigate ASC-US cytologically. STUDY DESIGN: Cytology samples from 129 patients diagnosed with ASC-US were used. Images of several atypical cells observed in these cases were presented on the Web. The study, based on the voting results, was presented and opinions were exchanged at the meeting of the Japanese Society of Clinical Cytology. RESULTS: The final diagnosis of ASC-US was benign lesions in 76 cases and low- and high-grade squamous intraepithelial lesions in 44, but no definite diagnosis could be made for the remaining 9. The total number of votes was 17,884 with a 36.5% consistency of cases judged as ASC-US. Benign cases were divided into 6 categories. Four categories not corresponding to the features of koilocytosis and small abnormal keratinized cells were judged as negative for an intraepithelial lesion or malignancy at a high rate. CONCLUSION: A Web-based survey would be useful which could be viewed at any time and thereby facilitate the sharing of cases to increase consistency.

Cytological features of lung adenocarcinoma with micropapillary pattern in the pleural or pericardial effusion: analysis of 5 cases.

Micropapillary pattern is a distinct histopathological pattern, and usually shows a high frequency of lymphatic invasion and lymph node metastases. This pattern is also reported in lung adenocarcinoma, however, only one cytological report of lung adenocarcinoma with micropapillary pattern has been reported. In this study, we analyzed the cytological features of this type of carcinoma in the pleural or pericardial effusion. This study was comprised of 5 consecutive cases of lung adenocarcinoma with micropapillary pattern, in which the tumor cells were present in the pleural or pericardial effusion and whose diagnoses were histopathologically confirmed. The characteristic cytological findings in the pleural or pericardial effusion were as follows: i) tightly cohesive small nests of tumor cells showing papillary structure without fibrovascular core, ii) these nests were comprised of approximately 5-20 tumor cells, iii) cauliflower-like and acinar-like structures were also observed, iv) intracytoplasmic vacuoles were observed in 40% of the cases, and v) the neoplastic cells had large round to oval nuclei containing coarse chromatin and occasional conspicuous nucleoli. It has been reported that the presence of micropapillary structure and intracytoplasmic vacuolation are also characteristic cytological features of micropapillary carcinoma of the urinary bladder, therefore, they are thought to be common cytological features of carcinomas with micropapillary pattern. Consequently, detection of these features can lead to a cytodiagnosis of lung adenocarcinoma with micropapillary pattern in the pleural or pericardial effusion. Recognition of these features is important because this type of tumor shows an aggressive clinical course.

Pigmented median raphe cyst of the penis with consideration of the possible mechanism of melanocytic colonization: A case report.

Median raphe cyst is a rare lesion located on the median raphe. The cyst wall is lined by cuboidal to columnar cells, transitional (urothelial) cells, stratified squamous cells or a mixture of these. The normal urethral mucosa and the median raphe cyst usually lack melanocytes and/or melanin pigment. However, albeit extremely rare, the presence of melanin pigment and/or melanocytes in median raphe cyst, namely pigmented median raphe cyst, has been previously reported. The current case report presents the sixth case of pigmented median raphe cyst and discusses the possible mechanism of melanocytic colonization in this tumor. A 48-year-old male presented with a nodule on the ventral surface of the penis. Histopathological study revealed that the cyst wall was covered by uniform bland cuboidal to urothelial cells. The peculiar observation was the presence of dendritic melanocytes among the epithelial cells. Therefore, a diagnosis of pigmented median raphe cyst was determined. Immunohistochemically, stem cell factor and endothelin-1 were not expressed in the epithelial cells of the cyst wall. It is well-known that melanocytes are rarely found in various non-melanocytic tumors, a phenomenon termed 'colonization'. The mechanism by which melanocytes appear in median raphe cyst remains unclear. The present report is the first to demonstrate that melanocytic proliferation and differentiation factors, such as stem cell factor and endothelin-1, are not involved in the pigmentation of median raphe cyst. In addition, aberrant melanocytic migration may contribute to the development of this type of lesion.

Signet-ring cell melanoma with sentinel lymph node metastasis: A case report with immunohistochemical analysis and review of the clinicopathological features.

Signet-ring cell melanoma is an extremely rare variant of malignant melanoma. A 68-year-old male presented with a black nodule on the left thigh. Histopathological examination revealed proliferation of sheet-like or variable-sized nests of atypical melanocytes. Neoplastic cells showing signet-ring cell appearance, characterized by the presence of eccentrically located enlarged nuclei and abundant pale cytoplasm, were also present. Immunohistochemically, the tumor cells were positive for S-100 protein, vimentin and Melan-A. Moreover, mammalian target of rapamycin (mTOR) pathway proteins were diffusely expressed. The current case report presents the 21st reported case of signet-ring cell melanoma. Analyses of the clinicopathological features revealed that this disease commonly affects middle-aged males and the presence of metastatic signet-ring cell melanoma with an unknown primary tumor. Immunohistochemical analyses of melanocytic markers have been useful for establishing the diagnosis of this type of disease, however, HMB-45 is occasionally found to be negative. In addition, the present case report is the first to analyze the expression of mTOR pathway proteins, which are central proteins involved in carcinogenesis and its inhibitor has been proposed as a therapeutic target for various types of tumor. Therefore, the mTOR inhibitor may also be a potential candidate for the treatment of this type of tumor.

Clear cell adenocarcinoma present exclusively within endometrial polyp: report of two cases.

Endometrial polyp is a common benign lesion that protrudes into the endometrial surface. The incidence of carcinoma within endometrial polyp is thought to be low, however, postmenopausal women with endometrial polyps are at an increased risk. Endometrial clear cell adenocarcinoma is a distinct and relatively rare subtype of endometrial carcinoma, and recent studies have proposed putative precursor lesions of clear cell adenocarcinoma, namely clear cell endometrial glandular dysplasia (EmGD) and clear cell endometrial intraepithelial carcinoma (EIC). Herein, we describe two cases of clear cell adenocarcinoma present exclusively within endometrial polyp and discuss the association of its precursor. Two postmenopausal Japanese females, 66-year-old (Case 1) and 54-year-old (Case 2) presented with abnormal genital bleeding. Cytological examination of both cases revealed adenocarcinoma, thus, hysterectomy was performed. Histopathological studies demonstrated clear cell adenocarcinoma within exclusively endometrial polyp in both cases. The peculiar finding in Case 1 was presence of atypical glandular cells with large round to oval nuclei and clear cytoplasm within the atrophic endometrial glands in the surrounding endometrial tissue, which corresponded to clear cell EIC. A recent study showed that 33% of uteri had at least one focus of clear cell EmGD in endometrial polyps. Accordingly, clear cell adenocarcinoma and clear cell EmGD can occur in association with endometrial polyps more frequently than previously thought. Therefore, detailed histopathological examination is important in diagnosis of endometrial polyps, especially in the postmenopausal women, moreover cytological examination is a useful tool in the postmenopausal women with endometrial polyps.

Cytological features of dedifferentiated adenoid cystic carcinoma of the trachea: a case report.

Adenoid cystic carcinoma (AdCC) is a distinct type of carcinoma, and cytological examination has been recognized as a useful tool in its diagnosis. Dedifferentiation is defined as the abrupt transformation of a low-grade tumor into a tumor with high-grade components. Albeit extremely rare, dedifferentiated AdCC has been reported: however, the cytological features of this tumor have not been documented. We observed a case in which a 66-year-old Japanese male had stenosis and thickness of the lower tracheal and bronchial walls. Cytological smears of a bronchial brush specimen revealed features typical for low-grade AdCC. However, a few cohesive epithelial cell clusters composed of large, atypical polygonal cells with large nuclei and conspicuous nucleoli also were present. This component was considered to represent dedifferentiated carcinoma. Histopathological study of the resected bronchial tumor revealed dedifferentiated AdCC. The cytological diagnosis of conventional low-grade AdCC is straightforward in most cases, although extremely rare, dedifferentiated carcinoma can occur within the conventional AdCC, and detection of a dedifferentiated component is possible in a cytological specimen because of obvious nuclear atypia. Therefore, careful observation is needed because cytologic diagnosis of dedifferentiated AdCC can help expedite treatment of this highly aggressive tumor.

Primary cutaneous B-cell lymphoma with abundant reactive gamma/delta T-cells within the skin lesion and peripheral blood.

T-cell/histiocyte-rich diffuse large B-cell lymphoma is characterized by abundant reactive T-cell and histiocyte infiltration within nodal diffuse large B-cell lymphoma, and only limited cases of primary cutaneous T-cell-rich B-cell lymphoma have been documented. These reactive T-cells usually show a T-helper phenotype. Gamma/delta T-cell is a functionally distinct T-cell lineage, which constitutes on average 5% of all T-cells in the peripheral blood. Herein, we report the first documented case of primary cutaneous malignant B-cell lymphoma with abundant reactive gamma/delta(+) T-cells within the skin lesion and peripheral blood. An 80-year-old Japanese male presented with a gradually enlarged knee nodule. Histopathological study revealed diffuse infiltration of lymphoid cells in the dermis and subcutis. Proliferation of large-sized atypical lymphoid cells was observed among medium-sized lymphocytes with convoluted nuclei. Immunohistochemically, these large-sized atypical lymphocytes were CD20(+), and relatively many gamma/delta(+) cell infiltration was also noted. Flowcytometric analysis revealed deviation of lambda+ cells (lambda/kappa 58) and increase of CD3(+) gamma/delta(+) cells (6%). Peripheral blood had CD3(+) gamma/delta(+) cells (28.8%). Rearrangement of immunoglobulin heavy chain, but not of T-cell receptor beta and gamma chains, was observed. Accordingly, an ultimate diagnosis of cutaneous B-cell lymphoma with abundant reactive gamma/delta(+) cells was made. Recent studies have shown reactive gamma/delta(+) T-cell infiltration and/or elevation in the peripheral blood in patients with various types of carcinoma, and that they play a role in the pathogenesis of some carcinomas. Therefore, additional analysis is needed to clarify the role of reactive gamma/delta(+) T-cells in malignant lymphoma.

Elastofibromatous change of the intestine: report of four lesions from three patients with review of the literature.

Elastofibromatous change, also referred to as elastofibromatous polyp or elastofibroma, has been extremely rarely described in the gastrointestinal tract. This lesion is characterized histopathologically by an excessive accumulation of elastic fibers occasionally with a fibrous component involving the submucosa and/or muscularis mucosae of the gastrointestinal tract. Herein, we report four additional lesions of the intestine and review the clinicopathological features of this rare lesion. Three patients (76-, 72-, and 52-year-old males) were detected with polypoid lesions in the jejunum, transverse and sigmoid colons, and sigmoid colon, respectively. All four lesions showed fundamentally the same histopathological and immunohistochemical features. The polypoid lesions were covered by non-neoplastic epithelium, and degenerated and truncated elastic fibers occasionally with a fibrous component had accumulated in the submucosa and/or muscularis mucosae. The characteristic feature was the elastofibromatous change centered around collections of elastotic submucosal vessels. Desmin-positive degenerative ruptured smooth muscle fibers were scattered within the elastic fibers in the submucosa. Our analyses of the clinicopathological features of the previously reported 32 cases of elastofibromatous change of the gastrointestinal tract as well as the present cases demonstrated that this type of lesion is most commonly found in the colon or rectum (29 cases), males, and middle-aged to elderly persons. Although the pathogenesis remains unclear, the convincing hypothesis that this lesion represents elastic degeneration of submucosal vessels by previous persistent vascular injury has been proposed. The collections of degenerative elastotic vascular walls may have an important role in the development of this lesion.

Small cell carcinoma of the urinary bladder and prostate: Cytological analyses of four cases with emphasis on the usefulness of cytological examination.

The occurrence of small cell carcinoma in the urinary bladder and prostate is rare. Only a few reports on the cytological features of small cell carcinoma of the urinary bladder in the urine specimen have been documented and, moreover, the urinary cytological features of prostate small cell carcinoma have been rarely reported. In this study, we analyzed the cytological features of four cases of small cell carcinoma of the urinary bladder and prostate, and discussed the usefulness of cytological examination of urine specimen for this type of tumor. This study included two urinary bladder and two prostate small cell carcinoma cases. Analyses of the cytological features of these cases revealed the following: i) the background was mostly inflammatory and necrotic material was also occasionally observed; ii) numerous tumor cells were present in two cases, whereas only a few neoplastic cells were observed in the remaining cases; iii) the neoplastic cells were small in size, had scant cytoplasm and a high nuclear/cytoplasmic ratio, and were arranged in small clusters or occasionally as single cells; iv) the tumor cell clusters showed prominent nuclear moldings; and v) the nuclei of the neoplastic cells were round to oval in shape with finely granular chromatin containing inconspicuous nucleoli. The cytological features of small cell carcinoma in the urine specimen are characteristic. Therefore, careful observation of the urine specimen may lead to a correct diagnosis of small cell carcinoma of the urinary bladder and, moreover, cytodiagnosis of prostate small cell carcinoma may also be possible.

Müllerianosis and endosalpingiosis of the urinary bladder: report of two cases with review of the literature.

Müllerianosis of the urinary bladder is an extremely rare benign condition, characterized by the presence of a mixture of at least two müllerian-derived components, and endosalpingiosis is also an extremely rare condition, characterized by the presence of tubal-type epithelium. In this report, we describe the 17(th) case of müllerianosis and 5(th) case of endosalpingiosis of the urinary bladder. A 39-year-old Japanese female presented with menstrual hematuria and was found to have a polypoid lesion in the posterior wall of the urinary bladder. Histopathological study demonstrated variably-sized dilated tubular glands in the lamina propria and muscularis propria. These dilated glands were covered by ciliated cuboidal cells, and some of them were covered by columnar cells with intracytoplasmic mucin. Moreover, a tiny focus of endometrial tissues was also present. Immunohistochemically, these glandular cells were positive for estrogen receptor. Accordingly, a diagnosis of müllerianosis was made. The second case was a 37-year-old Japanese female, who was found to have a polypoid lesion in the posterior wall of the bladder. Dilated tubular glands were covered by ciliated cells in the lamina propria and muscularis propria. Neither endocervical nor endometrial tissues were observed. Immunohistochemically, these ciliated cells were positive for estrogen receptor. Accordingly, a diagnosis of endosalpingiosis was made. Our analysis revealed that these two conditions mainly affect premenopausal females and occur exclusively in the posterior wall. Although the pathogenesis remains completely unresolved, a metaplastic theory is favored. The recognition of these two conditions is important because they can mimic invasive adenocarcinoma.

Mucinous carcinoma occurring in the male breast.

Male breast carcinoma is an uncommon neoplasm, accounting for 0.6% of all breast carcinomas. Invasive ductal carcinoma of no special type is the most common type of male breast carcinoma, and mucinous carcinoma occurring in the male breast is extremely rare. In the present study, we report a case of mucinous carcinoma of the male breast and discuss the clinicopathological features of this type of tumor. A 63-year-old Japanese male presented with a gradually enlarged nodule in the right breast. The resected breast specimen revealed pure mucinous carcinoma and immunohistochemical analyses demonstrated that tumor cells were positive for estrogen receptor (ER), but negative for progesterone receptor (PgR). In addition, HER2 expression was not amplified. Pure mucinous carcinoma is generally associated with a low incidence of lymph node or distant metastases, and excellent disease-free survival in females. However, certain cases of this type of tumor with axillary lymph node metastasis in the male breast have been reported. In addition, the immunoprofiles of mucinous carcinoma in males are fundamentally the same as those in females. More than 90% of cases show positive immunoreactivity for ER and/or PgR, and HER2 expression is not amplified. However, it has been reported that breast cancer in males is more frequently positive for ER than in females, and has less HER2 overexpression. The high rate of hormone receptor-positive breast cancer in males is considered to be due to similar conditions as those in breast cancer in postmenopausal women. The pathogenesis of male breast carcinoma, including mucinous carcinoma, remains unclear; therefore, additional clinicopathological studies are required.

Primary cutaneous anaplastic large cell lymphoma occurring in an atopic dermatitis patient: a case report with review of the literature with emphasis on their association.

Although the risk of malignant lymphoma in patients with atopic dermatitis (AD) remains controversial, an increased risk of malignant T-cell lymphoma in patients with AD has been reported. Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a relatively common distinct clinicopathological entity. However, occurrence of C-ALCL in patients with AD has been rarely reported. Herein, we describe the 5(th) reported case of C-ALCL occurring in a patient with AD and review the clinicopathological features. A 30-year-old Japanese male with a long-standing history of AD presented with a gradually enlarged nodular lesion in the right abdominal wall, which had spontaneously regressed without therapy. Two years later, multiple nodular lesions appeared in his trunk, and swelling of multiple lymph nodes was also detected. Histopathological studies demonstrated diffuse proliferation of large-sized lymphocytes with large convoluted nuclei containing conspicuous nucleoli and relatively rich cytoplasm in the skin and lymph node. Immunohistochemically, these lymphocytes were positive for CD30, CD8, and MUM1, and negative for CD3, CD4, and ALK1. Accordingly, a diagnosis of primary C-ALCL was made. The patient died of disease after various courses of chemotherapy. Our clinicopathological review revealed that the prognosis of C-ALCL occurring in patients with AD is poor because two of 5 patients died of disease. Therefore, albeit extremely rare, AD patients with C-ALCL should be monitored closely, and additional clinicopathological studies are needed to clarify the pathogenesis of C-ALCL occurring in patients with AD.

Sarcoidal granulomas in the mediastinal lymph nodes after treatment for marginal zone lymphoma of the esophagus: report of a case with review of the concept of the sarcoidosis-lymphoma syndrome.

Patients with sarcoidosis have a high risk of development of malignant lymphoma, and this association was coined the term "sarcoidosis-lymphoma syndrome". Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a distinct clinicopathological entity, and the stomach is the most common site. The occurrence of this type of lymphoma in the esophagus is extremely rare. In this report, we describe the first documented case of sarcoidal granulomas in the mediastinal lymph nodes after treatment for MALT lymphoma of the esophagus. A 60-year-old Japanese female was found to have a submucosal tumor in the esophagus. Histopathological study revealed proliferation of small- to medium-sized lymphoid cells with convoluted nuclei, and immunohistochemically, these lymphoid cells were diffusely positive for CD20, bcl-2, and MUM1. R-CHOP therapy was performed, which led to tumor remission. Two years later, swelling of the mediastinal lymph nodes was detected. Histopathological study of the lymph nodes revealed presence of variably-sized epithelioid granulomas without caseating necrosis but no malignant lymphoma was noted. Sarcoidal granulomas can be observed in patients with malignant tumors including malignant lymphoma and carcinoma without history of systemic sarcoidosis. It is important to recognize that systemic sarcoidosis and sarcoidal reaction without evidence of systemic disease can occur after development of malignant lymphoma, therefore, sarcoidal reaction must be included in the differential diagnostic consideration of recurrent malignant lymphoma.

Occurrence of Epstein-Barr virus-associated plasmacytic lymphoproliferative disorder after antithymocyte globulin therapy for aplastic anemia: a case report with review of the literature.

It is well established that patients with immunosuppression have a higher risk of development of lymphoproliferative disorders (LPDs), and Epstein-Barr virus (EBV) is associated with development of LPDs. Aplastic anemia (AA) is an immune-mediated hematological disorder, and immunosuppression therapy (IST), such as antithymocyte globulin (ATG), is widely used for treatment of AA. However, occurrence of LPD without bone marrow transplantation has been extremely rarely documented in patients with IST for AA. Herein, we report the 6th documented case of EBV-associated LPD after IST for AA and review the clinicopathological features of this extremely rare complication. A 46-year-old Japanese female was admitted for evaluation of progressive pancytopenia. Bone marrow biopsy revealed fatty marrow with marked decrease of trilineage cells, and bone marrow aspiration demonstrated no dysplastic changes. IST with rabbit ATG was administered, after which, she developed high fever. Bone marrow aspiration showed increase of atypical plasma cells with mildly enlarged nuclei and irregular nuclear contour. These atypical plasma cells were EBER-positive. Accordingly, a diagnosis of EBV-positive plasmacytic LPD was made. Most cases of LPDs are B-cell origin, and plasmacytic LPD is a rare subtype. The current report is the second case of plasmacytic LPD in patients with IST for AA. Therefore, detailed histopathological and immunohistochemical analyses are needed for correct diagnosis and treatment, and additional studies are needed to clarify the clinicopathological features of EBV-LPD after IST for AA.

Concomitant occurrence of IgG4-related pleuritis and periaortitis: a case report with review of the literature.

IgG4-related sclerosing disease is an established disease entity with characteristic clinicopathological features. Some recent reports have demonstrated that this disease can occur in the respiratory system including the pleura. Herein, we describe the first documented case of concomitant occurrence of IgG4-related pleuritis and periaortitis. A 71-year-old Japanese female with a history of essential thrombocythemia presented with persistent cough and difficulty in breathing. Computed tomography demonstrated thickening of the right parietal pleura, pericardium, and periaortic tissue and pleural and cardiac effusions. Histopathological study of the surgical biopsy specimen of the parietal pleura revealed marked fibrous thickening with lymphoplasmacytic infiltration. Phlebitis was noted, however, only a few eosinophils had infiltrated. Immunohistochemical study revealed abundant IgG4-positive plasma cell infiltration and high ratio of IgG4-/IgG-positive plasma cells (84%). Therefore, a diagnosis of IgG4-related pleuritis was made with consideration of the elevated serum IgG4 level (684 mg/dL). Recently, the spectrum of IgG4-related sclerosing disease has expanded, and this disease can occur in the pleura, pericardium, and periaortic tissue. Although histopathological analysis of the pericardium and periaortic tissue was not performed in the present case, it was suspected that thickening of the pericardium and periaortic tissue was clinically due to IgG4-related sclerosing disease. Our clinicopathological analyses of IgG4-related pleuritis and pericarditis reveal that this disease can present as dyspnea and pleural and pericardial effusion as seen in the present case, therefore, it is important to recognize that IgG4-related sclerosing disease can occur in these organs for accurate diagnosis and treatment.

Epstein-Barr virus-related post-transplant lymphoproliferative disorder occurring after bone marrow transplantation for aplastic anemia in Down's syndrome.

It is well established that Down's syndrome exhibits a predisposition to development of leukemia, however, association between aplastic anemia and Down's syndrome is exceptional. Herein, we describe a case of aplastic anemia occurring in Down's syndrome following post-transplant lymphoproliferative disorder (PTLD) after bone marrow transplantation (BMT). A 27-year-old Japanese male with Down's syndrome presented with a headache. Laboratory tests revealed severe pancytopenia, and bone marrow biopsy demonstrated hypocellular bone marrow with decrease of trilineage cells, which led to a diagnosis of aplastic anemia. One year after diagnosis, he was incidentally found to have an anterior mediastinal tumor, which was histopathologically diagnosed as seminoma. Subsequently, he received BMT from a female donor, and engraftment was observed. Three months after transplantation, he experienced cough and high fever. Biopsy specimen from the lung revealed diffuse proliferation of large-sized lymphoid cells expressing CD20 and EBER. These lymphoid cells had XY chromosomes. Thus, a diagnosis of EBV-associated PTLD was made. This is the seventh documented case of aplastic anemia occurring in Down's syndrome. Association between aplastic anemia and Down's syndrome has not been established, therefore, additional clinicopathological studies are needed. Moreover, this is the first case to undergo BMT for aplastic anemia in Down's syndrome. Although engraftment was observed, he developed EBV-positive PTLD. The neoplastic cells of the present case were considered to be of recipient origin, although the majority of PTLD cases with BMT are of donor origin.

Endometrioid adenocarcinoma concurrent with a blue nevus of the endometrium and uterine cervix: A case report.

A blue nevus is a benign melanocytic lesion that is composed of spindle-shaped pigmented melanocytes. Although the uterine cervix is believed to be the most common extracutaneous location of blue nevi, the occurrence of these lesions in the endometrial stroma has been reported, albeit rarely. The present study describes a case of endometrioid adenocarcinoma concurrent with a blue nevus of the endometrium and uterine cervix. A 58-year-old female presented with abnormal vaginal bleeding. A biopsy from the endometrium revealed an endometrioid adenocarcinoma and subsequently, a total hysterectomy was performed. Histopathological study revealed the proliferation of columnar cells that formed irregularly-shaped tubular and cribriform glands. The neoplastic columnar cells had large, round to oval nuclei containing a single small nucleolus. Focal squamous differentiation was noted. In the stroma of the non-neoplastic endometrium, single or small aggregates of short spindle-shaped cells containing melanin without atypia were observed. These melanocytes were also present in the endocervix. Therefore, the final diagnosis was of endometrioid adenocarcinoma concurrent with a blue nevus of the endometrium and cervix. This is the first documented case of a blue nevus of the endometrium and endocervix. The pathogenesis of blue nevi of the genital tract is not yet completely understood. Possible origins of these cells include Schwann cells or perineural cells of the peripheral nerve fiber or the abnormal migration of neural crest-derived cells.

Adult T-cell leukemia/lymphoma accompanying follicular mucinosis: a case report with review of the literature.

Follicular mucinosis is recognized as one of the histopathological reaction patterns characterized by the accumulation of mucin within follicular epithelium. It is induced by various causes including inflammatory diseases, and more than half of the cases are associated with malignant lymphoma, mainly mycosis fungoides. Herein, we describe the third documented case of adult T-cell leukemia/lymphoma (ATLL) accompanying follicular mucinosis. A 72-year-old Japanese male presented with persistent erythema in his arm and neck. Laboratory tests demonstrated positivity for human T-cell leukemia virus (HTLV)-1 antibodies. Histopathological study of the biopsy specimen from the neck revealed superficial perivascular, nodular, and intrafollicular lymphocytic infiltrations. These lymphocytes were small- to medium-sized and had convoluted nuclei. Mucoid material deposition was observed within the hair follicles, and it was digested by hyaluronidase. Immunohistochemically, these lymphocytes were positive for CD3, CD4, CD25, and Foxp3. Accordingly, an ultimate diagnosis of ATLL accompanying follicular mucinosis was made. The skin is the most common extralymphatic site of involvement of ATLL. The present case clearly demonstrated that albeit extremely rare, ATLL can cause follicular mucinosis. Therefore, ATLL should be included in the differential diagnostic consideration of follicular mucinosis.