Olfactory neuroblastoma associated with extensive "in situ" lesion and aberrant glandular and rhabdomyosarcomatous differentiation.

A case of olfactory neuroblastoma (ONB) associated with extensive intraepithelial neoplastic proliferation, evidenced by an "in situ" lesion, in the overlying olfactory epithelium and aberrant glandular and rhabdomyosarcomatous differentiation is reported. The tumor was a polypoid lesion that involved the upper nasal cavity and ethmoid sinus of a 63-year-old woman and consisted of an ONB surrounded by and mixed with a proliferative lesion of rhabdomyoblastic cells, consistent with an embryonal rhabdomyosarcoma. A few small foci of tubular glands with mucus-producing cells were also observed. In the olfactory epithelium covering the polypoid lesion, a nested or band-like arrangement of primitive-appearing small cells was found, and the tumor cells were immunoreactive for epithelial cell adhesion molecule (detected with Ber-EP4) and low-molecular weight cytokeratin (detected with CAM5.2) but not for synaptophysin or calretinin. The intraepithelial lesion was contiguous with the subepithelial cell nests of ONB and appeared to invade the subjacent stroma and show transition to ONB, and some tumor cell nests of ONB also contained small aggregates of similar primitive-appearing cells. The intraepithelial growth was considered to represent a preinvasive precursor lesion of ONB. Previous descriptions of an "in situ" lesion in ONB are limited. The aberrant glandular and rhabdomyosarcomatous differentiation noted in this case is also an exceptionally rare phenomenon of ONB.

[Prognostic analysis of patients with T1 stage high grade of bladder urothelial carcinoma and glandular differentiation].

Objective: To evaluate the recurrence and progression of patients with pT1 high grade urothelial carcinoma of bladder (UCB) and glandular differentiation. Methods: We retrospectively analyzed the clinical and pathological information of 208 patients diagnosed as pT1 high grade urothelial carcinoma in the Fifth Central Hospital of Tianjin from January 2006 to February 2019.Among them, 78 cases were diagnosed as glandular differentiation (UCGD), the other 130 patients without histologic variants were served as control. The UCGD group included 62 male and 16 female, whose median age was 67 years old (range 38-81 years old). The control group contained 105 male and 25 female, whose median age was 66 years old (range 40-82 years old). Kaplan-Meier and Cox proportional hazard regression analyses were used to evaluate the predictors of oncologic outcomes. Results: The disease recurrence rate and progression rate in UCGD group were 65.4% (51/78) and 28.2% (22/78), higher than 38.5%(50/130) and 14.6%(19/130) of control group (P<0.05). The median recurrence time in UCGD group was 41 months while 55 months in the control group. The median progression time in UCGD group was 39 months while 54 months in the control group. According to the univariate analysis, largest tumor size (P=0.030), UCGD (P=0.003) and lymphovascular invasion (LVI) (P=0.032) were associated with disease recurrence. UCGD (P=0.036) and LVI (P=0.011) were associated with progression. Additionally, Cox multivariate analysis revealed that UCGD (P=0.001), LVI (P=0.038) were the independent factors of disease recurrence. UCGD (P=0.007) and LVI (P=0.037) were also found to be the independent factors of disease progression. Conclusions: Patients with T1 stage UCB and UCGD are at higher risk of disease recurrence and progression. Therefore, these patients should be followed up closely after being diagnosed and undergo individual treatment according to the situation.

Retroperitoneal low grade endometrial stromal sarcoma with florid endometrioid glandular differentiation: Cytologic-histologic correlation and differential diagnosis.

Low grade endometrial stromal sarcoma (LGESS) is a rare neoplasm that typically arises in the uterine corpus and accounts for less than 1% of uterine sarcomas. Infrequently, extra-uterine LGESS can occur. Histologically, LGESS is characterized by a monotonous population of cells that resemble the proliferative phase of endometrial stroma and in their classic form they exhibit tongue-like growth pattern of infiltration and/or lymphovascular invasion. Infrequently LGESS can demonstrate various morphologic differentiation patterns, including endometrioid-type glands. We report the first fine needle aspiration (FNA) case of a periduodenal mass that was incidentally discovered on Computed Tomography (CT) scan of a 60-year-old female. The cytomorphologic and histologic findings and the immunohistochemical staining were consistent with a LGESS with endometrioid glandular differentiation. We are presenting the correlation between the cytologic, radiologic and pathologic features.

A Rare Presentation of Recurrent Malignant Peripheral Nerve Sheath Tumor with Glandular Differentiation-A Case Report.

Malignant peripheral nerve sheath tumors (MPNST) are rare heterogeneous group of soft tissue neoplasms. In most cases, they originate within the pre-existing neurofibromatosis. The emergence of glandular structures in MPNST is curious and enigmatic. We report a case of recurrent MPNST with glandular differentiation arising in the background of neurofibroma in a 20-year-old lady. By immunohistochemistry, MPNST showed focal positive staining for S100 and negative staining for SOX10 while adjacent neurofibroma showed diffuse positivity for S100 and SOX10. The glandular tumor cells showed positive staining for CDX2, Cam5.2, CK19, and CK7 (focal), while negative for SOX10 and S100. MPNST with glandular differentiation is quite rare which may pose a diagnostic challenge. The glandular differentiation in MPNST should be excluded from the metastasis from second primary with the aid of clinical and radiological correlation.

Impact of Trinal Histological Glandular Differentiation Grade on the Prognosis of Patients with Intrahepatic Cholangiocarcinoma: a Multicenter Retrospective Study.

INTRODUCTION: It is unclear whether the histological glandular differentiation (HGD) score that evaluates the tumor grade of two dominant components is prognostic for survival in patients with intrahepatic cholangiocarcinoma (ICC). METHOD: We retrospectively analyzed the clinical and histopathologic data of 235 consecutive patients with histologically confirmed ICC following hepatectomy at 5 university hospitals in the Kansai region of Japan. RESULTS: Survival was statistically significantly stratified by trinal HGD grade (p < 0.05). Median disease-free survival (DFS) of patients with high HGD grade was significantly shorter compared with moderate HGD grade (13.0 vs 31.2 months, respectively; p = 0.004). By Cox proportional hazards regression analysis, HGD grade had the fifth-highest hazard ratio (HR = 1.77, p = 0.002) for DFS after vascular and/or biliary invasion, extrahepatic invasion, lymph node metastasis and multiple tumors. Multivariate logistic regression analysis revealed four predictors of early recurrence after hepatectomy (lymph node metastasis: odds ratio [OR] = 3.74, p = 0.001; tumor size > 50 mm: OR = 2.80, p = 0.002; HGD grade, high: OR = 2.11, p = 0.012; and vascular or biliary tract invasion: OR = 2.11, p = 0.048). CONCLUSION: Trinal HGD grade had a significant prognostic impact on the survival of patients with ICC after radical hepatectomy.

Bladder preservation in complicated invasive urothelial carcinoma following treatment with cisplatin/gemcitabine plus tislelizumab: A case report.

BACKGROUND: Invasive urothelial carcinoma (UC) with squamous and glandular differentiation is a highly malignant and complicated pathological subtype, and the standard care is radical cystectomy (RC). However, urinary diversion after RC significantly reduces patient quality of life, thus bladder-sparing therapy has become a research hotspot in this field. Recently, five immune checkpoint inhibitors have been approved for systemic therapy of locally advanced or metastatic bladder cancer by the Food and Drug Administration, but the efficacy of immunotherapy combined with chemotherapy for invasive UC is still unknown, especially for pathological subtypes with squamous and glandular differentiation. CASE SUMMARY: We report the case of a 60-year-old male who complained of repetitive painless gross hematuria and was diagnosed with muscle-invasive bladder cancer with squamous and glandular differentiation, defined as cT3N1M0 according to the American Joint Committee on Cancer, who had a strong desire to preserve the bladder. Immunohistochemical staining revealed that programmed cell death-ligand 1 (PD-L1) expression in the tumor was positive. Thus, a transurethral resection to maximize removal of the bladder tumor was performed under cystoscopy, and the patient subsequently received a combination of chemotherapy (cisplatin/gemcitabine) and immunotherapy (tislelizumab) treatment. No tumor recurrence in the bladder was observed following pathological and imaging examination after 2 cycles and 4 cycles of treatment, respectively. The patient achieved bladder preservation and has been tumor-free for more than two years. CONCLUSION: This case shows that the combination of chemotherapy and immunotherapy might be an effective and safe treatment strategy for PD-L1 expression positive UC with divergent histologic differentiation.

Squamous or Glandular Differentiation Predicts Poor Prognosis in pT1 Urothelial Carcinoma.

BACKGROUND: The aim of this study was to evaluate the prognosis of patients with T1N0M0 urothelial carcinoma with squamous differentiation (UCSD) or glandular differentiation (UCGD) because it has not been determined whether these variant histologies behave more aggressively than pure urothelial carcinoma (PUC). PATIENTS AND METHODS: Ninety-nine patients diagnosed with pT1N0M0 bladder cancer and treated conservatively with transurethral resection of bladder tumor at Kanazawa University Hospital between 2007 and 2019 were included in this study. The overall survival, cancer-specific survival (CSS), and recurrence-free survival of the variant histology and PUC groups were evaluated and compared. RESULTS: The variant histology group had significantly lower overall survival (p=0.006) and CSS (p=0.0095) than the PUC group did. Variant histology was found to be an independent prognostic factor in univariate and multivariate analyses for overall survival and CSS. On the other hand, no significant difference in progression-free survival was observed between the two groups (p=0.439). However, the variant histology group had significantly lower overall survival (p=0.004) and CSS (p=0.004) after progression. CONCLUSION: The prognosis for patients with pT1 bladder cancer with UCSD or UCGD treated conservatively with transurethral resection of bladder tumor was poor. Considering the worse prognosis of these patients after stage progression, early radical cystectomy could be recommended.

Survival of Patients With UrAC and Primary BAC and Urothelial Carcinoma With Glandular Differentiation.

Purpose: To investigate the significance of demographic and pathological characteristics on the survival outcomes of urachal adenocarcinoma (UrAC), primary bladder adenocarcinoma (BAC) and urothelial carcinoma with glandular differentiation (UCGD) in China. Materials and Methods: We retrospectively analyzed cases with non-distant metastases (≤ T4M0). Of 106 patients, 30 (28.3%), 40 (37.7%), and 36 (34.0%) met the criteria for UrAC, primary BAC, and UCGD, respectively. Data on patient demographics, tumor pathology, and survival outcomes were collected. The median follow-up was 36 months. Survival was analyzed using multivariate Cox regression. Results: Patients with UrAC were younger (51.87 ± 15.25 years) than those with primary BAC (60.50 ± 12.56 years) and UCGD (63.83 ± 11.60 years) (P<0.001). Patients with UrAC were the most likely to be stage T3-4 (70.0% vs. 40.0% vs. 44.4%; P<0.001), while the primary BAC group had a higher rate of poor differentiation than the UrAC and UCGD groups (57.4% vs. 18.5% vs. 24.1%; P<0.001). The Kaplan-Meier curves showed that the overall survival (OS), progression-free survival (PFS), and disease-specific survival (DSS) of the primary BAC group were poorer than those of both the UrAC and UCGD groups (P=0.0046,P<0.0001,P=0.0077 respectively). Regarding BAC, patients with mucinous adenocarcinoma tended to have better OS and PFS than those with other histological types (P<0.005,P=0.0245). Multivariate Cox regression analysis revealed that tumor type (P=0.002), T stage (P=0.034), and the age-adjusted Charlson Comorbidity Index (aCCI) scores (P=0.005) predicted the postoperative OS and DSS of the patients. For PFS, the tumor type (P=0.011), grade (P=0.000), and aCCI (P=0.002) scores were predictive. Conclusion: Among UrAC, primary BAC, and UCGD patients, the prognosis was poorest for those with primary BAC. Attempts should be made to diagnose these aggressive tumors early, since patients in whom tumors are detected early appear to survive longer.

A case of esophageal squamous cell carcinoma with neuroendocrine, basaloid, and ciliated glandular differentiation.

Esophageal carcinomas have multidirectional differentiation abilities and different histological components have been reported. Herein, we report a case of esophageal carcinoma with four different differentiations. A 64-year-old man was referred to our hospital for treatment of an esophageal tumor detected during an esophagogastroduodenoscopy, which revealed an elevated lesion accompanied by a slightly depressed lesion in the middle of the esophagus. Examination of the biopsy specimen obtained from the elevated lesion revealed an adenocarcinoma, while that from the depressed lesion revealed a squamous cell carcinoma. Fluorodeoxyglucose-position emission tomography and enhanced computed tomography showed an esophageal carcinoma in the middle of the esophagus with no signs of metastasis. The preoperative diagnosis was adenosquamous cell carcinoma classified as T2N0M0 according to the TNM classification (seventh edition). Thoracoscopic esophagectomy was performed. Examination of the resected specimen revealed esophageal squamous cell carcinoma with neuroendocrine, basaloid, and ciliated glandular differentiation. Although they may be totipotent, an esophageal carcinoma consisting of four components is extremely rare. Moreover, ciliated glandular differentiation is rarely observed in the esophagus, except in individuals with bronchial esophageal duplication cysts and adenocarcinoma arises from a Barrett's esophagus.

Comparative genomic profiling of glandular bladder tumours.

Primary glandular bladder tumours (bladder adenocarcinoma [BAC], urachal adenocarcinoma [UAC], urothelial carcinoma with glandular differentiation [UCg]) are rare malignancies with histological resemblance to colorectal adenocarcinoma (CORAD) in the majority of this subgroup. Definite case numbers are very low, molecular data are limited and the pathogenesis remains poorly understood. Therefore, this study was designed to complement current knowledge by in depth analysis of BAC (n = 12), UAC (n = 13), UCg (n = 11) and non-invasive glandular lesions (n = 19). In BAC, in addition to known alterations in TP53, Wnt, MAP kinase and MTOR pathway, mutations in SMAD4, ARID1A and BRAF were identified. Compared to published data on muscle invasive bladder cancer (BLCA) and CORAD, UCg exhibited frequent "urothelial" like alterations while BAC and UAC were characterised by a more "colorectal" like mutational pattern. Immunohistochemically, there was no evidence of DNA mismatch repair deficiency or PD-L1 tumour cell positivity in any sample. Depending on the used antibody 0-45% of BAC, 0-30% of UCg and 0% UAC cases exhibited PD-L1 expressing tumour associated immune cells. A single BAC (9%, 1/11) showed evidence of ARID1A protein loss, and two cases of UCg (20%, 2/10) showed loss of SMARCA1 and PBRM1, respectively. Taken together, our data suggest at least in part involvement of similar pathways driving tumourigenesis of adenocarcinomas like BAC, UAC and CORAD independent of their tissue origin. Alterations of TERT and FBXW7 in single cases of intestinal metaplasia further point towards a possible precancerous character in line with previous reports.

Carcinogen-induced bladder cancer in the FVB mouse strain is associated with glandular differentiation and increased Cd274/Pdl-1 expression.

BACKGROUND: Creation of genetically engineered mouse models of bladder cancer often involves the use of several background strains in conjunction with the carcinogen N-butyl-N-(4-hydroxybutyl) nitrosamine (BBN). However, carcinogen susceptibility in commonly used strains, as well as phenotypic differences is not well characterized. OBJECTIVES: To determine differences in susceptibility and phenotypic outcome following BBN exposure of C57BL/6 and FVB, two strains commonly used for model development. METHODS: Male C57BL/6 and FVB mice were exposed to BBN (0.05%) in drinking water for 12 and 16 weeks. Dissected bladders were characterized by histological and immunohistochemical analyses. Gene Ontology analysis was performed to identify differences in gene expression across strains following BBN exposure. RESULTS: While the C57BL/6 strain developed non-invasive tumors, FVB mice developed muscle invasive bladder cancer with squamous and/or glandular differentiation. Glandular differentiation was exclusively observed in the FVB strain. FVB tumors were highly immunogenic and inflamed by the presence of high expression of Cd274 (Pdl-1), murine histocompatibility complex (H2) and pro-inflammatory cytokines (Il-5 and Il-17). CONCLUSIONS: Following BBN exposure, FVB mice undergo rapid tumorigenesis and disease progression characterized by Pdl-1 expression and development of glandular differentiation. These studies identify a degree of tumor heterogeneity in the FVB tumors previously undescribed, and identify FVB mice as a potentially useful model for the study of bladder adenocarcinoma and the inflammatory tumor microenvironment.

Primary intrafascial desmoplastic melanoma with pseudoglandular differentiation and aberrant cytokeratins expression: An exceptional presentation.

Desmoplastic melanoma (DM) is an uncommon variant of malignant melanoma (MM), histologically characterized by a mainly dermal proliferation of spindled cells within a desmoplastic stroma. Normally, involvement of deeper tissues by DM is the result of direct extension down from the overlying dermis. MM is widely known to harbor a striking potential for morphological and phenotypic variability; among MM morphological variants, pseudoglandular MM is characterized by extensive discohesion within cords and nests of malignant cells and ensuing formation of so-called pseudolumina, thus mimicking adenocarcinoma. We present an exceptional case of DM characterized by intrafascial origin, partly pseudoglandular differentiation, and aberrant experession of cytokeratins in the pseudoglandular component; genetic data from next-generation sequencing supported the final diagnosis of DM, as well as the ontogenetic identity of the pseudoglandular component. Prior to this report, pseudoglandular features had never been described in DM. Additionally, our case is unusual because of the deep origin of the tumor, arising below the subcutaneous fat of the scalp, as well as the aberrant experession of cytokeratins in the pseudoglandular component, thus posing a challenging differential diagnosis with several soft tissue tumors.

Malignant Peripheral Nerve Sheath Tumor With Divergent Glandular Differentiation.

Malignant peripheral nerve sheath tumors (MPNST) are soft tissue neoplasms with evidence of nerve sheath differentiation. They usually arise from peripheral nerves or from preexisting benign nerve sheath neoplasms, often in patients with neurofibromatosis type 1 (NF1). The histologic diagnosis of MPNST is challenging as their morphology is highly variable, and there has been a lack of routine diagnostic immunohistochemical markers and specific genetic aberrations. Although divergent differentiation is well documented in MPNST, it is most frequently toward mesenchymal elements. Differentiation toward epithelial elements is very rare, and we illustrate a case of MPNST with glandular differentiation, comprising prominent well-formed glands, with a brief discussion of biphasic (spindle and glandular) neoplasms in the differential diagnosis. An index of suspicion for MPNST is necessary, due to the differing management from tumors in its differential diagnosis, and because of the potential for therapies toward molecular targets in future.

Metastatic Urothelial Carcinoma with Glandular Differentiation That Confirmed the Response by Autopsy Specimen to Second-Line mFOLFOX6 (Fluorouracil, Oxaliplatin, and Leucovorin) plus Bevacizumab Chemotherapy.

The prognostic significance of glandular differentiation in urothelial carcinoma (UC) is controversial, and thus far there is no established treatment strategy against metastasis of glandular component. We describe here a case of metastatic UC with glandular differentiation that had histological disappearance of adenocarcinoma components at autopsy after sequential chemotherapy with S-1 and cisplatin (CDDP) and with mFOLFOX6 (fluorouracil, oxaliplatin, and leucovorin) plus bevacizumab (mFOLFOX6+Bev). A 62-year-old Asian male was diagnosed with invasive UC with glandular differentiation (T2N0M0) by radical cystectomy and ileal conduit, and careful follow-up observation was made. Eight years after radical operation, peritoneal metastases occurred, and a biopsy specimen using colon fiber revealed high-grade adenocarcinomas with an immunohistochemical profile that included positivity for cytokeratin 7 (CK7) and negativity for cytokeratin 20 (CK20) and uroplakin, which was identical to the radical cystectomy specimen. Thus, he received combination chemotherapy consisting of S-1 and CDDP; however, the peritoneal metastasis worsened after 2 cycles. Therefore, second-line mFOLFOX6+Bev chemotherapy was performed for a total of 5 courses. In spite of this, the patient died, and the final diagnosis by autopsy was multiple metastases of infiltrating pure UC to the lung, bone, and peritoneum. Interestingly, there were no pathological findings of adenocarcinoma, and the immunohistochemical profile of the metastatic lesions was identical to that of the previous specimens from the bladder and colon. This suggests that sequential chemotherapy of S-1 and CDDP and second-line mFOLFOX6+Bev might be a feasible option in metastatic UC with glandular differentiation.

Role of SATB2 in distinguishing the site of origin in glandular lesions of the bladder/urinary tract.

The differential diagnosis of glandular lesions of the bladder/urinary tract can be challenging because of significant morphologic and immunohistochemical overlap between primary lesions and metastasis/direct extension from adjacent organs. Special AT-rich sequence-binding protein 2 (SATB2), encoded on chromosome 2q32-33, is a recently described DNA-binding protein involved in osteoblast lineage commitment and expressed in colorectal and appendiceal neoplasms. In this study, we hypothesized that immunohistochemistry for SATB2 may be of value in distinguishing primary adenocarcinoma of the bladder/urinary tract and urothelial carcinoma with glandular differentiation from gastrointestinal and endocervical primaries. Intensity and distribution of SATB2 nuclear labeling were semiquantitatively scored and compared with those of CDX2. The study included 43 primary adenocarcinomas of the bladder/urinary tract, 20 urothelial carcinomas with glandular differentiation, 26 adenocarcinomas of the uterine cervix, and 22 colorectal adenocarcinomas involving the bladder. Positive SATB2 immunostaining was observed in 21 of 43 (49%) primary bladder/urinary tract adenocarcinomas, in 17 of 22 (77%) colorectal adenocarcinomas, and in the glandular component of 4 of 18 (22%) urothelial carcinomas with glandular differentiation. SATB2 was negative in 25 of 26 endocervical adenocarcinomas and showed focal weak immunostaining (1+) in 1 of 26 (4%). The results were not significantly different from those seen with CDX2. We conclude that SATB2 immunohistochemistry is not useful in supporting urothelial versus gastrointestinal or endocervical origin in the differential diagnosis of glandular lesions of the bladder/urinary tract.

Glandular differentiation in dedifferentiated chondrosarcoma: molecular evidence of a rare phenomenon.

Epithelial glandular differentiation in dedifferentiated chondrosarcoma has not been described. Our patient was a 64-year-old man with a history of prostate cancer status post-radiation and hormonal therapy. On screening bone scan, he was found to have increased uptake in his right femoral shaft. Biopsy revealed intermediate-grade conventional chondrosarcoma. Subsequent femoral resection was remarkable for an intermediate-grade chondrosarcomatous component juxtaposed to an area composed of anastomosing nests and cords of malignant epithelial cells showing nuclear atypia and increased mitotic activity. A fibroblastic-appearing spindle cell population was intimately associated with the epithelial cells. The epithelial cells labeled with 34bE12, AE1/AE3, EMA, and Vimentin (both spindled and epithelial components) while being negative for prostate-specific antigen, prostate specific acid phosphatase, cytokeratin 20, thyroid transcription factor-1, and CDX2. The patient developed local recurrence 9 months after the initial resection but has had no metastatic disease and consistently undetectable prostate-specific antigen levels. Deep parallel sequencing of the dedifferentiated component showed a nonsynonymous mutation at exon 4 of IDH1 gene at codon R132 leading to a substitution of arginine, with serine confirming glandular differentiation in dedifferentiated chondrosarcoma.

Thymomas with prominent glandular differentiation: a clinicopathologic and immunohistochemical study of 12 cases.

Twelve cases of thymomas with prominent glandular differentiation are presented. The patients were 7 men and 5 women aged between 45 and 68 years (average, 56.5 years). Clinically, the patients presented with nonspecific symptoms of chest pain, cough, and fatigue. None of the patients had a history of myasthenia gravis or other autoimmune syndrome. Thymectomy was performed in all patients. The tumor size ranged from 4 to 7 cm in greatest diameter. Macroscopically, the tumors were described as firm and light tan without areas of necrosis, hemorrhage, or cystic change. Histologically, 7 tumors were classified as spindle cell (World Health Organization type A), 2 as mixed spindle cell and conventional (A+B1), 2 as conventional (B1), and 1 as atypical thymoma (B3). In 4 cases, the tumors showed invasion into periadipose thymic tissue. All cases showed the typical growth patterns of their particular subtypes. In addition, a distinct glandular component was present in all cases showing mucinous differentiation in 4 of them. Immunohistochemical studies showed tumor cells positive for CAM5.2, cytokeratin 5/6, and Pax8 and negative for carcinoembryonic antigen, thyroid transcription factor 1, and epithelial membrane antigen. Calretinin showed focal weak staining in the nonmucinous glandular components in 3 cases. Follow-up information obtained in 8 patients showed that all were alive and well in a period ranging from 2 to 5 years. The possibility of a glandular component in thymomas should be kept in mind in the assessment of mediastinoscopic biopsies to avoid misdiagnosis for other neoplasms that may require different treatment modalities.

Right atrial myxoma with glandular differentiation: A rare entity in pediatric age group.

Cardiac myxomas (CMs) account for nearly half of the primary cardiac tumors in the elderly. They arise from sub-endocardial "reserve" or lepidic" cells, which may show divergent differentiation. We describe a CM with glandular differentiation in the right atrium of a 10-year-old child who presented with respiratory distress on exertion, of 2 months duration. On echocardiography, two large interconnected masses measuring 34×30 mm and 20×17 mm were seen to arise from the free wall of the right atrium. Cut surface of the excised mass was myxoid with areas of calcification. On microscopy, there were typical features of a myxoma with prominent glandular differentiation and characteristic immunophenotype. The case is being reported due to its rarity in pediatric age group as well as its glandular differentiation, which must be recognized as a spectrum of histomorphologic diversity and must not be mistaken for a metastatic adenocarcinoma.