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International Journal of Surgery Case... 2014Central papillary cystadenocarcinoma of the jaw is an extremely rare tumor with only three previously reported cases in the English literature. This tumor is a...
INTRODUCTION
Central papillary cystadenocarcinoma of the jaw is an extremely rare tumor with only three previously reported cases in the English literature. This tumor is a histologically low-grade cancer, affecting more commonly in the mandible than in the maxilla.
PRESENTATION OF CASE
A 65-year-old woman presented with a two months history of a rapidly growing, painless mass of the right ascending ramus of the mandible. The pathologic report from incisional biopsy was a papillary cystic tumor with a differential diagnosis of cystadenoma versus cystadenocarcinoma. Segmental mandibulectomy, parotidectomy and submandibular gland resection were performed. The final pathology was intraosseous papillary cystadenocarcinoma.
DISCUSSION
Clinical features of central papillary cystadenocarcinoma of the mandible mimic an odontogenic lesion and metastatic bone disease, careful review of radiograph and pathology should be done. Surgical excision with wide margins is the appropriate treatment. Postoperative radiation therapy should be considered in histologically aggressive or high-stage tumor.
CONCLUSION
This is the fourth case of central papillary cystadenocarcinoma of the mandible in the English literature. Although it is usually a low-grade cancer, en bloc resection with adjuvant postoperative radiotherapy in a high-stage disease, and long-term follow-up allow the patient to have a favorable prognosis.
PubMed: 24794027
DOI: 10.1016/j.ijscr.2014.04.006 -
Taiwanese Journal of Obstetrics &... Jun 2014
Topics: Cicatrix; Cystadenocarcinoma, Papillary; Cystadenocarcinoma, Serous; Female; Humans; Laparoscopy; Ovarian Cysts; Peritoneal Neoplasms; Postoperative Complications
PubMed: 25017255
DOI: 10.1016/j.tjog.2014.04.002 -
Autopsy & Case Reports 2022Papillary cystadenocarcinoma of the salivary gland is a very rare malignant neoplasm accounting for only 2% of all salivary gland lesions. In 1991 it was first included... (Review)
Review
Papillary cystadenocarcinoma of the salivary gland is a very rare malignant neoplasm accounting for only 2% of all salivary gland lesions. In 1991 it was first included as a separate entity in the World Health Organization (WHO) classification of salivary gland tumors and in 2017 WHO Classification, the tumor was clubbed as a sub-variant of adenocarcinoma, not otherwise specified. It most commonly occurs in the major salivary glands. Herein we report a case of salivary papillary cystadenocarcinoma in a 54-year-old female, who presented with rapid enlargement of the right parotid swelling. Based on radiology and fine-needle aspiration cytology, a working diagnosis of the malignant tumor involving the superficial lobe of the right parotid gland was made. In view of the malignant nature of the swelling, superficial parotidectomy was done. The histopathology and immunohistochemistry of the mass confirmed the diagnosis of papillary cystadenocarcinoma of the right parotid. With the revised 2017 WHO classification of salivary gland tumors, it is important to report all rare subtypes in order to understand their biology and behavior.
PubMed: 35252049
DOI: 10.4322/acr.2021.357 -
Journal of Oral Biology and... 2017A 68-year-old Caucasian gentleman presented with a 6-month history of a left sided Level I/II neck swelling involving the floor of mouth. MRI revealed a large cystic...
A 68-year-old Caucasian gentleman presented with a 6-month history of a left sided Level I/II neck swelling involving the floor of mouth. MRI revealed a large cystic lesion and histology confirmed a diagnosis of primary papillary cystadenocarcinoma of the sublingual gland. Papillary cystadenocarcinoma was first described in 1991 by the World Health Organisation [WHO], and is a rare malignant neoplasm characterised by cysts and papillary endo-cystic projections. Papillary cystadenocarcinoma arising from the sublingual glands is extremely rare and has the potential to metastasise to cervical lymph nodes. This patient we report was therefore treated with surgical excision and post-operative radiotherapy.
PubMed: 29124004
DOI: 10.1016/j.jobcr.2017.03.003 -
Genome Medicine May 2020Distinct prevalence of inherited genetic predisposition may partially explain the difference of cancer risks across ancestries. Ancestry-specific analyses of germline...
BACKGROUND
Distinct prevalence of inherited genetic predisposition may partially explain the difference of cancer risks across ancestries. Ancestry-specific analyses of germline genomes are required to inform cancer genetic risk and prognosis of diverse populations.
METHODS
We conducted analyses using germline and somatic sequencing data generated by The Cancer Genome Atlas. Collapsing pathogenic and likely pathogenic variants to cancer predisposition genes (CPG), we analyzed the association between CPGs and cancer types within ancestral groups. We also identified the predisposition-associated two-hit events and gene expression effects in tumors.
RESULTS
Genetic ancestry analysis classified the cohort of 9899 cancer cases into individuals of primarily European (N = 8184, 82.7%), African (N = 966, 9.8%), East Asian (N = 649, 6.6%), South Asian (N = 48, 0.5%), Native/Latin American (N = 41, 0.4%), and admixed (N = 11, 0.1%) ancestries. In the African ancestry, we discovered a potentially novel association of BRCA2 in lung squamous cell carcinoma (OR = 41.4 [95% CI, 6.1-275.6]; FDR = 0.002) previously identified in Europeans, along with a known association of BRCA2 in ovarian serous cystadenocarcinoma (OR = 8.5 [95% CI, 1.5-47.4]; FDR = 0.045). In the East Asian ancestry, we discovered one previously known association of BRIP1 in stomach adenocarcinoma (OR = 12.8 [95% CI, 1.8-90.8]; FDR = 0.038). Rare variant burden analysis further identified 7 suggestive associations in African ancestry individuals previously described in European ancestry, including SDHB in pheochromocytoma and paraganglioma, ATM in prostate adenocarcinoma, VHL in kidney renal clear cell carcinoma, FH in kidney renal papillary cell carcinoma, and PTEN in uterine corpus endometrial carcinoma. Most predisposing variants were found exclusively in one ancestry in the TCGA and gnomAD datasets. Loss of heterozygosity was identified for 7 out of the 15 African ancestry carriers of predisposing variants. Further, tumors from the SDHB or BRCA2 carriers showed simultaneous allelic-specific expression and low gene expression of their respective affected genes, and FH splice-site variant carriers showed mis-splicing of FH.
CONCLUSIONS
While several CPGs are shared across patients, many pathogenic variants are found to be ancestry-specific and trigger somatic effects. Studies using larger cohorts of diverse ancestries are required to pinpoint ancestry-specific genetic predisposition and inform genetic screening strategies.
Topics: Ataxia Telangiectasia Mutated Proteins; BRCA2 Protein; Fanconi Anemia Complementation Group Proteins; Female; Genetic Predisposition to Disease; Germ-Line Mutation; Humans; Male; Neoplasms; RNA Helicases; Risk Factors; Succinate Dehydrogenase; Von Hippel-Lindau Tumor Suppressor Protein
PubMed: 32471518
DOI: 10.1186/s13073-020-00744-3 -
Modern Pathology : An Official Journal... Jan 2016Serous carcinoma (SC) represents ~10% of endometrial carcinomas, but is responsible for almost 40% of cancer deaths. This article reviews the main pathological features,... (Review)
Review
Serous carcinoma (SC) represents ~10% of endometrial carcinomas, but is responsible for almost 40% of cancer deaths. This article reviews the main pathological features, differential diagnosis, and the usefulness of molecular pathology and immunohistochemistry in its diagnosis. Most helpful features for the diagnosis include: irregularly shaped and sized papillae, slit-like spaces, cell stratification and budding, highly atypical cells, architectural and cytological discordance in pseudoglandular tumors, as well as lack of endometrioid features. SC shows typically a predominant papillary growth, which is also found in some subtypes of endometrioid carcinoma of the endometrium (EEC). Distinction is easy when attention is paid to the presence of diffuse marked nuclear pleomorphism, but also to the complex papillary architecture. SC may also show a solid or pseudoglandular patterns, and in these cases differential diagnosis may be difficult with EEC grade 3. Moreover, a high proportion of SC may exhibit clear cells, and, thus, may be confused with clear cell carcinoma. Finally, it is sometimes difficult to distinguish mixed SC-EEC, from SC that combines papillary and pseudoglandular growths. Although there is not a single immunohistochemical marker for distinguishing SC from its mimickers, some antibodies are useful (p53, p16, IMP2, and IMP3), particularly when used in combination. Diagnosis of SC may be even more problematic in small biopsies; a diagnosis of high-grade endometrial carcinoma, SC component can not be excluded, is acceptable as a managerial approach, so it could be taken into account at the time of final surgery.
Topics: Cystadenocarcinoma, Serous; Endometrial Neoplasms; Female; Humans
PubMed: 26715173
DOI: 10.1038/modpathol.2015.141 -
Surgical Oncology Clinics of North... Apr 2016Management of cystic neoplasms of the pancreas is challenging as it relies on radiologic and cyst fluid markers to discriminate between benign and pre-cancerous lesions,... (Review)
Review
Management of cystic neoplasms of the pancreas is challenging as it relies on radiologic and cyst fluid markers to discriminate between benign and pre-cancerous lesions, however their ability to predict malignancy is limited. While asymptomatic serous cystadenomas can be managed conservatively, mucinous cystic neoplasms and intraductal papillary mucinous neoplasms are more difficult to manage. A selective approach, based on the preoperative likelihood of high-grade dysplasia or invasive disease, is the standard of care. Research is focusing on the development of pre-operative markers for identifying high risk lesions, which will spare patients with low-risk or benign lesions the risks of pancreatectomy.
Topics: Cystadenocarcinoma, Mucinous; Cystadenoma, Mucinous; Cystadenoma, Serous; Humans; Pancreatic Cyst; Pancreatic Neoplasms
PubMed: 27013369
DOI: 10.1016/j.soc.2015.11.006 -
Archives of Pathology & Laboratory... Jan 2007Primary peritoneal serous borderline tumor is a rare epithelial proliferation that can present as an incidental finding at laparotomy and raises concern for a primary... (Review)
Review
CONTEXT
Primary peritoneal serous borderline tumor is a rare epithelial proliferation that can present as an incidental finding at laparotomy and raises concern for a primary ovarian tumor with peritoneal implants.
OBJECTIVE
To present a brief history of this condition and describe its distinctive histology and clinical presentation, as well as to review the chief differential diagnostic considerations, to include mesothelial proliferations, endosalpingiosis, endometriosis, high-grade primary peritoneal papillary serous carcinoma, and implants from primary ovarian serous neoplasms.
DATA SOURCES
Relevant articles indexed in PubMed (National Library of Medicine) between 1966 and 2005, references thereof, and reference surgical pathology texts.
CONCLUSIONS
Primary peritoneal serous borderline tumor should be considered in the differential diagnosis of an epithelial proliferation with prominent psammoma bodies on the peritoneal surface of specimens submitted for nongynecologic complaints.
Topics: Cystadenocarcinoma, Serous; Cystadenoma, Serous; Diagnosis, Differential; Female; Humans; Male; Peritoneal Neoplasms
PubMed: 17227115
DOI: 10.5858/2007-131-138-PPSBT -
Journal of Surgical Oncology Jan 2013Pancreatic cancer is a genetic disease. Pancreatic cancers develop from one of three precursor lesions, pancreatic intraepithelial neoplasia (PanIN), intraductal... (Review)
Review
Pancreatic cancer is a genetic disease. Pancreatic cancers develop from one of three precursor lesions, pancreatic intraepithelial neoplasia (PanIN), intraductal papillary mucinous neoplasms (IPMNs), and mucinous cystic neoplasms (MCNs), and each arises in association with distinct genetic alterations. These alterations not only provide insight into the fundamental origins of pancreatic cancer but provide ample opportunity for improving early diagnosis and management of cystic precursors.
Topics: Carcinoma in Situ; Carcinoma, Pancreatic Ductal; Cell Transformation, Neoplastic; Chromogranins; Cystadenocarcinoma, Mucinous; DNA-Binding Proteins; Disease Progression; GTP-Binding Protein alpha Subunits, Gs; Humans; Mutation; Oncogene Proteins; Pancreatic Neoplasms; Ubiquitin-Protein Ligases
PubMed: 22806689
DOI: 10.1002/jso.23213 -
Head and Neck Pathology Jul 2013Low-grade salivary duct carcinoma (LG-SDC) is a rare neoplasm characterized by predominant intraductal growth, luminal ductal phenotype, bland microscopic features, and... (Review)
Review
Low-grade salivary duct carcinoma (LG-SDC) is a rare neoplasm characterized by predominant intraductal growth, luminal ductal phenotype, bland microscopic features, and favorable clinical behavior with an appearance reminiscent of florid to atypical ductal hyperplasia to low grade intraductal breast carcinoma. LG-SDC is composed of multiple cysts, cribriform architecture with "Roman Bridges", "pseudocribriform" proliferations with floppy fenestrations or irregular slits, micropapillae with epithelial tufts, fibrovascular cores, and solid areas. Most of the tumor cells are small to medium sized with pale eosinophilic cytoplasm, and round to oval nuclei, which may contain finely dispersed or dark condensed chromatin. Foci of intermediate to high grade atypia, and invasive carcinoma or micro-invasion have been reported in up to 23 % of cases. The neoplastic cells have a ductal phenotype with coexpression of keratins and S100 protein and are surrounded by a layer of myoepithelial cells in non-invasive cases. The main differential diagnosis of LG-SDC includes cystadenoma, cystadenocarcinoma, sclerosing polycystic adenosis, salivary duct carcinoma in situ/high-grade intraductal carcinoma, and papillary-cystic variant of acinic cell carcinoma. There is no published data supporting the continuous classification of LG-SDC as a variant of cystadenocarcinoma. Given that most LG-SDC are non-invasive neoplasms; the terms "cribriform cystadenocarcinoma" and LG-SDC should be replaced by "low-grade intraductal carcinoma" (LG-IDC) of salivary gland or "low-grade intraductal carcinoma with areas of invasive carcinoma" in those cases with evidence of invasive carcinoma.
Topics: Biomarkers, Tumor; Carcinoma in Situ; Humans; Immunohistochemistry; Neoplasm Grading; Salivary Ducts; Salivary Gland Neoplasms
PubMed: 23821212
DOI: 10.1007/s12105-013-0460-1