Primary Mucinous Adenocarcinoma Research Articles

The Utility of Preoperative Tumor Markers in Peritoneal Carcinomatosis from Primary Appendiceal Adenocarcinoma: an Analysis from the US HIPEC Collaborative

BackgroundPrognostication based on preoperative clinical factors is lacking in patients undergoing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC). This study aims to determine the value of preoperative tumor markers as predictors of progression-free survival (PFS) and overall survival (OS) for patients with peritoneal carcinomatosis from a primary mucinous adenocarcinoma of the appendix (MACA). MethodsWe queried the United States HIPEC Collaborative, a database of patients with peritoneal carcinomatosis treated with CRS/HIPEC at twelve institutions between 2000 and 2017, identifying 409 patients with MACA. Multivariate analysis was used to identify independent predictors of disease progression. Subgroup analysis was conducted to evaluate the impact of tumor grade on the predictive value of tumor markers. ResultsCA19-9 [HR 2.44, CI 1.2–3.4] emerged as an independent predictor of PFS while CEA [HR 4.98, CI 1.06–23.46] was independently predictive of OS (p <0.01). Tumor differentiation was the most potent predictor of both PFS (poorly differentiated vs well, [HR 4.5 CI 2.01–9.94]) and OS ([poorly differentiated vs well-differentiated: [HR 13.5, CI 3.16–57.78]), p <0.05. Among patients with combined CA19-9 elevation and poorly differentiated histology, 86% recurred within a year of CRS/HIPEC (p < 0.01). Similarly, the coexistence of CEA elevation and unfavorable histology led to the lowest survival rate at two years [36%, p < 0.01]. CA-125 was not predictive of PFS or OS. ConclusionElevated preoperative CA19-9 portends worse PFS, while elevated CEA predicts worse OS after CRS/HIPEC in patients with MACA. This study provides additional evidence that CA19-9 and CEA levels should be collected during standard preoperative bloodwork, while CA-125 can likely be omitted. Tumor differentiation, when added to preoperative tumor marker levels, provides powerful prognostic information. Prospective studies are required to confirm this association.

17847 A critical appraisal of the history of Mohs micrographic surgery and its evolution in the management of primary mucinous adenocarcinoma

Primary mucinous carcinoma (PMC) of the skin is a rare head and neck malignancy. It is resistant to chemotherapy and radiation; therefore, standard treatment includes surgical excision with 1–2-cm margins. Mohs micrographic surgery (MMS) is fast becoming the criterion standard for management of cutaneous carcinomas, offering maximal tissue conservation and high cure rates. We present a brief review of the evolution of MMS, highlight changing practice patterns and introduce a new therapeutic option for PMC. MMS was developed in the 1930s by Frederic E. Mohs. Initially referred to as “chemosurgery,” Dr. Mohs implemented fixed tissue techniques using a chemical cauterant, zinc chloride (ZnCl). He discovered that tissue injected with ZnCl remained histologically preserved. He microscopically examined horizontal sections of fixed tissue from various tumors thereby developing the basic premise of MMS. In 1936, he successfully treated the first squamous cell carcinoma of the lower lip using MMS. The technique has since evolved and is currently applied to various cutaneous malignancies. Our extensive PubMed literature review revealed that the majority of PMC cases are treated with traditional surgical excision (85.5%) and only few with MMS (9.4%). The first case of PMC treated with MMS was published in 1988 with only 20 cases reported in total. Published data reveals lower recurrence rates of PMC treated with MMS (0%-7%) versus excision (30%-40%). In conclusion, MMS appears to be superior to standard excision for PMC and should be considered as first line therapy for primary and recurrent lesions especially in cosmetically sensitive areas.

Primary Thymic Mucinous Adenocarcinoma: A Case Report Focusing on Radiological Findings and Review of the Literature

: Primary thymic mucinous adenocarcinoma is a very rare disease. Its clinical findings are not well known. Its prognosis is poor. We present a case of primary thymic mucinous adenocarcinoma in a 60-year-old woman. The patient visited the emergency room with dysarthria as a chief complaint, and a mass was found in the occipital bone and petrous apex in brain computed tomography (CT) and magnetic resonance imaging (MRI). The lesion was considered metastasis, and body CT evaluation was performed to find the primary origin. In CT, a large peripheral enhancing mass with central low attenuation and calcification was found in the anterior mediastinum. Pathologic confirmation of the anterior mediastinal mass was done by percutaneous needle biopsy procedure. The patient was diagnosed with primary thymic mucinous adenocarcinoma. We also reviewed and analyzed previous literature focusing on radiologic findings. The possibility of primary thymic mucinous adenocarcinoma should be considered if a large mass with a heterogeneous enhancement pattern is accompanied by adjacent tissue involvement, associated thymic cyst, or calcification.

Managing a Rare Case of Primary Mucinous Adenocarcinoma of the Vagina at a Tertiary Cancer Center

Background: Vaginal cancer is one of the rarest of malignancies and accounts for only 1%–2% of all gynecologic malignancies. An intestinal-type, primary mucinous adenocarcinoma is extremely rare, a...

Molecular Genetic Features of Primary Nonurachal Enteric-type Adenocarcinoma, Urachal Adenocarcinoma, Mucinous Adenocarcinoma, and Intestinal Metaplasia/Adenoma: Review of the Literature and Next-generation Sequencing Study

The diagnosis of primary adenocarcinoma of the urinary bladder may be challenging in routine practice. These tumors may morphologically and immunohistochemically overlap with urachal adenocarcinoma and colorectal adenocarcinoma. Further, their genetic background is poorly understood. We systematically searched the PubMed database for results of complex genetic evaluation of primary bladder adenocarcinoma subtypes. Subsequently, we designed our own series of bladder lesions. We evaluated 36 cases: 16 primary enteric-type adenocarcinomas, 7 urachal enteric adenocarcinomas, 3 primary mucinous/colloid adenocarcinomas, and 10 intestinal-type metaplasia/villous adenoma. Detailed clinical data were collected, and all cases were examined using targeted next-generation sequencing. On the basis of the literature, the first mutated gene in these tumors was reported to be KRAS in 11.3% of cases, followed by TERT promoter mutations in 28.5%. In addition to KRAS and TERT, other genes were also found to be frequently mutated in primary bladder adenocarcinoma, including TP53, PIK3CA, CTNNB1, APC, FBXW7, IDH2, and RB1. In our series, the most frequent gene mutations in primary enteric-type adenocarcinomas were as follows: TP53 (56%); BRCA2, KMT2B (both 33%); NOTCH2, KDR, ARID1B, POLE, PTEN, KRAS (all 28%); in urachal enteric adenocarcinoma they were as follows: TP53 (86%); PTEN, NOTCH (both 43%); in primary mucinous/colloid adenocarcinomas they were as follows: KRAS, GRIN2A, AURKB (all 67%); and, in intestinal-type metaplasia/villous adenoma, they were as follows: APC, PRKDC (both 60%); ROS1, ATM, KMT2D (all 50%). No specific mutational pattern was identified using cluster analysis for any of the groups. Herein, we describe the pathologic features and immunohistochemical staining patterns traditionally used in the differential diagnoses of glandular lesions of the bladder in routine surgical pathology. We outline the mutational landscape of these lesions as an aggregate of published data with additional data from our cohort. Although diagnostically not discriminatory, we document that the most common genetic alterations shared between these glandular neoplasms include TP53, APC (in the Wnt pathway), and KRAS (in the MAPK pathway) mutations.

A Tertiary Cancer Center Experience of 52 Cases of Primary Ovarian Mucinous Adenocarcinomas.

Background Primary mucinous epithelial ovarian adenocarcinoma (mEOC) constitutes a small percentage (2–5%) of ovarian cancer. Our aim is to understand the clinicopathological characteristics and survival results of patients with mEOC after a long-term follow-up. Materials and Methods This is a retrospective study of primary mEOC cases treated at a tertiary cancer center in India, from January 1, 2005, to December 31, 2012. Results Out of 958 malignant ovarian tumors, 52 (5.43%) were mucinous adenocarcinoma. Nearly 71.2% of cases were of early-stage disease, and the remaining were of advanced-stage disease. After a follow-up period of 63 months (range: 1–138 months), the 5-year actuarial overall survival for stages I, II, III, and IV was 92.5, 70, 38.5, and 0%, respectively. Among advanced-stage tumors, half of them progressed without undergoing cytoreductive surgery and died. Conclusion Most of the mEOC cases present in early stages and have good clinical outcome. Patients with advanced-stage disease do not respond well to standard chemotherapy regimens in use and have poor survival figures. The use of primary cytoreduction should be considered in the place of interval cytoreduction for advanced mEOC.

Primary mucinous adenocarcinoma of the renal pelvis misdiagnosed as calculous pyonephrosis: a case report and literature review

Primary mucinous adenocarcinoma of the renal pelvis is a rare malignant disease that is difficult to diagnose preoperatively. There are still no characteristic symptoms, radiological features, or standard treatment for this tumor with only ~100 cases reported. The prognosis is poor. We report a case of a 66-year-old man who presented with a 2-month history of fever and right waist pain. He was misdiagnosed with calculous pyonephrosis and underwent percutaneous nephrostomy (PCN) at a local hospital. Gelatinous material was drained via a PCN catheter. He was then transferred to our hospital. He had elevated CEA and CA19-9. We performed an open radical nephrectomy and found polypoid, gelatinous material and stones filling the renal pelvis. He was diagnosed with primary mucinous adenocarcinoma of the renal pelvis by pathology. He refused adjuvant chemotherapy and there was no sign of recurrence after one year of follow-up. By assessing a literature review of all of the cases reported since 2000, we recommend that careful history taking, serum tumor markers, and CT scans may improve the diagnostic accuracy rates and radical nephrectomy with total ureterectomy accompanied by adjuvant therapy may improve the prognosis.

MUCINOUS ADENOCARCINOMA OF GALL BLADDER: A RARE ENTITY IN GALL BALDDER ADENOCARCINOMA

Gall bladder carcinoma is the most common type of tumor of the biliary tract and adenocarcinoma is the major histopathological subtype. Mucinous variant is a rare subtype and mucinous carcinoma accompanied with signet ring is extremely rare. When mucinous component exceeds 90% of the tumor, it is labeled as pure mucinous carcinoma. Immunohistochemistry helps to distinguish primary mucinous adenocarcinoma of gall bladder from pseudo myxoma peritonei from appendiceal neoplasm. Mucinous carcinoma is CK 7 positive and CK20 negative. We report a case of mucinous adenocarcinoma arising from the fundus of the gall bladder.&#x0D; Keywords: Mucinous carcinoma, Gall bladder, Adenocarcinoma, Metastatic deposits

PRIMARY HIGH-GRADE MUCINOUS ADENOCARCINOMA OF THE RENAL PELVIS AND URETER: A CASE REPORT AND SYSTEMATIC REVIEW

Introduction: Tumor of the renal pelvis was uncommon, as well as the primary mucinous adenocarcinoma of the upper urinary tract. This was aggressive cancer and had a poor prognosis compared with urothelial carcinoma. Methods: This study presented a 55-year-old male with primary high-grade mucinous adenocarcinoma of the upper urinary tract. From the initial assessment until 8 months of follow up after surgery and adjuvant chemotherapy. A systematic review of the last 20-year-literatures was also performed. There were 32 case reports, with 34 cases reported worldwide. Results: The majority of the subjects with primary mucinous adenocarcinoma were older male, presented with flank pain, and had a long history of urinary tract stone. The standard surgical modalities were radical nephrectomy, total ureterectomy, and bladder cuff excision. In most cases, only radical nephrectomy was carried out, because the histopathological results were obtained after surgery. The recurrence and distant metastases were widely reported. Conclusion: The finding of primary mucinous adenocarcinoma of the upper urinary tract was rare and proven after the histopathological finding of the surgical specimen. The treatment modalities were approached with radical nephrectomy, total ureterectomy, followed by chemotherapies with or without radiotherapy.

Primary Invasive Mucinous Adenocarcinoma of the Lung: Prognostic Value of CT Imaging Features Combined with Clinical Factors.

ObjectiveTo investigate the association between CT imaging features and survival outcomes in patients with primary invasive mucinous adenocarcinoma (IMA).Materials and MethodsPreoperative CT image findings were consecutively evaluated in 317 patients with resected IMA from January 2011 to December 2015. The association between CT features and long-term survival were assessed by univariate analysis. The independent prognostic factors were identified by the multivariate Cox regression analyses. The survival comparison of IMA patients was investigated using the Kaplan-Meier method and propensity scores. Furthermore, the prognostic impact of CT features was assessed based on different imaging subtypes, and the results were adjusted using the Bonferroni method.ResultsThe median follow-up time was 52.8 months; the 5-year disease-free survival (DFS) and overall survival rates of resected IMAs were 68.5% and 77.6%, respectively. The univariate analyses of all IMA patients demonstrated that 15 CT imaging features, in addition to the clinicopathologic characteristics, significantly correlated with the recurrence or death of IMA patients. The multivariable analysis revealed that five of them, including imaging subtype (p = 0.002), spiculation (p < 0.001), tumor density (p = 0.008), air bronchogram (p < 0.001), emphysema (p < 0.001), and location (p = 0.029) were independent prognostic factors. The subgroup analysis demonstrated that pneumonic-type IMA had a significantly worse prognosis than solitary-type IMA. Moreover, for solitary-type IMAs, the most independent CT imaging biomarkers were air bronchogram and emphysema with an adjusted p value less than 0.05; for pneumonic-type IMA, the tumors with mixed consolidation and ground-glass opacity were associated with a longer DFS (adjusted p = 0.012).ConclusionCT imaging features characteristic of IMA may provide prognostic information and individual risk assessment in addition to the recognized clinical predictors.

Primary Mucinous Adenocarcinoma of Appendix Invading Urinary Bladder with a Fistula: A Case Report.

An adenocarcinoma of the appendix invading the urinary bladder, which is difficult to be diagnosed before the operation, is an extremely rare disease. Only a few cases have been reported. Here we reported a case of patient diagnosed with the mucinous adenocarcinoma of the appendix invading the urinary bladder. The case reported in this study was a 54-years old man who was admitted due to a 6-month history of intermittent episodes of irritative voiding symptoms of the bladder, and weight loss. The patient did not have any gastrointestinal symptoms. The physical examination, laboratory examination, cytology of the urine, computed tomography and cystoscopy were inconclusive. The partial cystectomy, subsequent exploratory laparotomy and intraoperative frozen analysis revealed the appendiceal mucinous adenocarcinoma with a fistula to the urinary bladder. The appendectomy and the right hemicolectomy with a ileocolic anastomosis, the lymphadenectomy and the partial cystectomy limited to the anterior wall was performed. Six months after operation, the patient was in a good health with no obvious discomfort, no recurrence or distant metastases. The recommended treatment for the adenocarcinoma of the appendix invading the bladder with a fistula formation is as follows: appendectomy, right hemicolectomy with ileocolic anastomosis, lymphadenectomy, partial cystectomy and intraperitoneal hyperthermic chemoperfusion.

Analysis of the clinicopathological characteristics, genetic phenotypes, and prognostic of pure mucinous adenocarcinoma.

BackgroundPrimary pure mucinous adenocarcinoma of the lung (PMA) is a rare subtype. However, correlations between clinicopathological features and genetic phenotypes with survival have not been described comprehensively.MethodsPure mucinous adenocarcinoma patient information collected from the Surveillance, Epidemiology, and End Results (SEER) database, the Department of Thoracic Surgery, Zhongshan Hospital, Fudan University (FDZSH), and the Cancer Genome Atlas (TCGA) were extracted, evaluated, and compared with other lung adenocarcinomas (LUAD) patient data. Gene Ontology and Kyoto Encyclopedia of Genes and Genomes pathway analyses were performed to explore the functional importance of underlying molecular changes. Overall survival (OS) was evaluated with the Kaplan‐Meier method. Univariate and multivariate analysis through Cox proportional hazard regression identified risk factors that predicted OS, and the results were used to construct a nomogram to predict OS for PMA patients.ResultsOverall, 3622 patients, 41 patients, and 15 patients with PMA were identified from the SEER, FDZSH, and TCGA databases, respectively. There were 345 differentially expressed genes, 30 differentially mutated genes and 72 differentially methylated genes were identified between PMA and other LUAD samples. In the SEER database, PMA had a better prognosis compared to other LUAD. Compared with patients with other LUAD, patients with PMA exhibited unique clinicopathological features, including fewer grade III/IV tumors, less pleural invasion, more early‐stage cancer, and more lower lobe carcinomas. Multivariate analyses showed that age, race, T stage, N stage, surgery, and chemotherapy were independent risk factors. The nomogram had a calibration index of 0.724.ConclusionsOur research identified unique clinicopathological characteristics and genetic phenotypes for PMA and other LUAD. The nomogram accurately predicted OS.

Primary mucinous adenocarcinoma of renal pelvis (case description)

This is a description of a case of extremely rare pathology, a primary mucinous adenocarcinoma of renal pelvis. The survey data, including CT-scan, as well as the results of histological, immunohistochemical and molecular genetic studies are presented. The disease progressed after surgical treatment; chemotherapy regimens and their effectiveness are given.

Survival of patients with resected primary colorectal mucinous adenocarcinoma: A competing risk nomogram analysis.

The aim of the present study was to use a competing risk model to analyze the prognostic value of mucinous adenocarcinoma (MAC) in patients with colorectal cancer (CRC). An additional aim was to construct nomograms for estimating the 3- and 5-year overall survival (OS) and cancer specific survival (CSS) rates of patients with primary CRC with MAC. The data were extracted from the Surveillance, Epidemiology, and End Results database, and a Multivariate Cox model and competing risk model were applied to assess the OS and CSS. Cox-based and competing risk-based nomograms were constructed and internally validated by discrimination and calibration, using the bootstrapping method with 1,000 times replicates. A total of 13,035 MAC and 61,958 non-mucinous adenocarcinoma (NMAC) CRC patients were enrolled in the present study. Compared with NMAC, MAC patients had a poorer OS and CSS time in the overall population, and in subgroups that comprised metastatic, non-metastatic, male, site of sigmoid colon, rectosigmoid junction and rectal CRC cases (HR>1; P<0.05). The Cox and competing risk-based nomograms showed effective discrimination and calibration. In conclusion, MAC was associated with poor OS and CSS in patients with CRC of the distal colon and rectum. The nomograms of primary CRC patients with MAC may aid the identification of individual patients with a high risk of overall mortality and cancer-associated mortality within 3 or 5 years.

Primary Mucinous Adenocarcinoma: The Critical Role of Morphology in a Rare Neoplasm

Abstract Objectives Primary mucinous adenocarcinoma of the skin is a rare clinical entity derived primarily from sweat glands with reported sites mainly arising from the head and neck region. Difficulty then arises with distinguishing between these primary neoplasms and those with similar morphology in the breast and/or gastrointestinal tract. Presentation of Case We report a case of an 85-year-old female with a past medical history of endometrial cancer presenting to clinic with a slow-growing right temporal lesion that was excised and diagnosed as primary mucinous adenocarcinoma. Further clinical workup and imaging revealed an oral lesion diagnosed as squamous cell carcinoma after excision along with an enhancing right breast mass with biopsy and excision showing an invasive ductal carcinoma with papillary and micropapillary features. This unusual presentation of more than two malignancies provides a challenge to differentiate between primary versus metastatic disease, especially with a rare malignancy that is present. Discussion A review of the literature on primary mucinous adenocarcinoma of the skin is presented, discussing epidemiology, clinical presentation, morphology, and immunohistochemistry sets historically studied to aid in diagnosis, with our case relying on morphology to finalize our diagnosis. Conclusion This unusual case highlights the difficulty in differentiating between primary versus metastatic mucinous adenocarcinoma yet highlights the value of morphology to aid in arriving at a diagnosis to help guide correct clinical treatment and follow-up.

Primary vaginal adenocarcinoma of intestinal-type: case report of a rare gynaecological tumour.

ABSTRACTVaginal cancer is rare and accounts for only 1 to 2% of all gynaecological malignancies. They arise as primary squamous cell cancers or are the result of extension from the cervix or vulva. Primary mucinous vaginal adenocarcinoma of intestinal-type is an extremely rare malignancy of unknown histogenesis with a diagnostic dilemma for the clinician and histopathologist. We presented the case of a 40-year-old Para 0+2 woman with the complaint of a mass in the vagina and recurrent vaginal bleeding who was evaluated and worked-up for examination under anaesthesia and biopsy of the vaginal mass. The histological examination revealed the unusual intestinal-type variant of adenocarcinoma of the vagina. Recognition of this rare entity is important, particularly to avoid the pitfall of misdiagnosing metastatic disease as primary vaginal cancer.

Comparing clinicopathological features and prognosis of primary pulmonary invasive mucinous adenocarcinoma based on computed tomography findings

ObjectiveTo evaluate the relationship between clinicopathologic characteristics and prognosis in patients with invasive mucinous adenocarcinoma (IMA) of the lung.MethodsA total of 68 patients who underwent surgical resection for primary lung IMA were reviewed during the period of 2009 and 2017. Tumors were classified as solitary-type or pneumonic-type according to the computed tomography (CT) findings. Cox proportional hazards model was used to assess the effects of clinicopathological characteristics on univariate and multivariable analyses of disease-free survival (DFS).ResultsSolitary-type was found in 54 patients, while pneumonic-type was found in 14 patients. The patients’ age varied between 56 and 68 years (patients’ median age was 61 years). Besides, 50 patients had T1/T2 tumor stage (73.5%). Compared with solitary-type, higher T stage, N stage, and pathological stage (P < 0.001) were found in pneumonic-type. Moreover, the survival analysis showed that the pneumonic-type had a significantly poorer DFS compared with solitary-type (P = 0.004). Univariate analysis showed that pneumonic pattern on CT scan, T stage, pathologic stage, and thyroid transcription factor-1 (TTF-1) were significant predictive factors of survival (P = 0.011, 0.014, 0.013, 0.029, respectively). Multivariate analysis further indicated that pneumonic-type was the only independent prognostic factor for poor survival [hazard ratio (HR) = 6.764, 95% confidence interval (CI): 1.563–29.269, P = 0.011].ConclusionsBased on CT findings, the solitary-type IMA is associated with a lower stage and better prognosis compared with the pneumonic-type IMA.

Clinicopathological analysis of breast primary mucinous cyst adenocarcinoma

目的： 探讨乳腺原发性黏液性囊腺癌（mucinous cystadenocarcinoma，MCA）的临床病理特征及鉴别诊断。 方法： 分析青岛大学附属医院2018年3月收集的1例MCA的组织形态学及免疫表型资料。 结果： 乳腺肿物切面部分区域呈胶冻样。镜下见大小不等充满黏液的囊腔，囊壁内衬富含黏液的柱状细胞，单层或复层，或形成复杂分支的乳头状，不同区域细胞表现不同程度的异型性。肿瘤细胞细胞角蛋白（CK）7、GATA3阳性，CK20阴性，雌激素受体（ER）、孕激素受体（PR）、HER2均阴性。Ki-67阳性指数60%。大部分囊壁周围肌上皮细胞p63及SMMHC阴性。 结论： 乳腺原发MCA是罕见肿瘤，其形态与卵巢、胰腺或胃肠道黏液性囊腺癌类似，诊断应首先排除转移的可能。MCA具有高Ki-67阳性指数和三阴性免疫表型，但其预后良好。.

Disseminated and massive tumor burden in a case of primary thymic mucinous adenocarcinoma

Primary thymic mucinous adenocarcinoma is an extremely rare type of thymic carcinoma that is resistant to radiation and chemotherapy. We present a case of primary thymic mucinous adenocarcinoma with disseminated metastases and rapid progression, after concurrent chemoradiation and platinum-based doublet chemotherapy. Case and literature review also revealed poor outcomes for primary thymic mucinous adenocarcinoma patients receiving surgery and adjuvant therapy. We concluded the poor response of either resectable or unresectable primary thymic mucinous adenocarcinoma.

Primary Mucinous Eccrine Adenocarcinoma: A Rare Skin Adnexal Tumor at an Unusual Site.

Primary Mucinous Eccrine Adenocarcinoma: A Rare Skin Adnexal Tumor at an Unusual Site.