Mural Nodule Of Anaplastic Carcinoma Research Articles

Metastatic Hepatobiliary Cystadenocarcinoma Mimicking a Gynecologic Malignancy

Hepatobiliary cystadenocarcinoma is a rare malignancy associated with ovarian-like stroma and overtly malignant glands that has a favorable prognosis if completely resected, analogous to minimally invasive adenocarcinoma arising in mucinous cystic neoplasms of the pancreas. Ovarian mucinous neoplasms may have a similar histologic appearance and are known to occasionally contain mural nodules of anaplastic carcinoma. We report a case of hepatobiliary cystadenocarcinoma that subsequently presented as metastatic disease mimicking a primary gynecological malignancy. The 30-year-old patient had a history of a completely resected hepatobiliary cystadenocarcinoma 2 years prior to presentation, without adjuvant therapy. She presented to her gynecologist with pelvic pain and vaginal bleeding. Physical examination revealed diffuse nodularity along the anterior vaginal wall, and imaging showed a 5.8 × 4.1 cm pelvic mass with diffuse metastatic disease. Biopsies of the vaginal wall and an inguinal lymph node showed nests of pleomorphic cells with squamoid and glandular features concerning for metastatic ovarian carcinoma; however, immunohistochemistry was negative for PAX8 and ER, but positive for CDX2. Subsequent review of the patient's prior resection revealed a cystic mucinous neoplasm with mural nodules of adenosquamous and anaplastic carcinoma, the latter previously reported only rarely in hepatobiliary cystadenocarcinoma. This case highlights the histologic overlap between hepatobiliary and ovarian neoplasms, which may present a diagnostic challenge, particularly in the setting of incomplete history. In addition, the unusual presence of an anaplastic component in the patient's original tumor portends a worse prognosis; therefore, additional therapy should be considered in these patients.

An Ominous Sign: Mucinous Ovarian Carcinoma with Sister Mary Joseph Nodule

Despite two centuries of progress in its surgical and oncological management, ovarian cancer remains the most lethal of the gynaecological cancers, claiming the lives of nearly 185,000 women globally each year. Historically considered a single disease, there is growing recognition that ovarian cancer is in fact a spectrum of malignancies with distinct cellular origins, molecular driver pathways and clinicopathological features. Mucinous ovarian carcinoma (mOC) is a rare histological subtype that presents a particular challenge in accurate diagnosis and management. Frequently confused with metastatic deposits from extra-ovarian mucinous tumours, the true incidence of primary mOC is estimated to be between 3-5%. Typically affecting younger women, prognosis for late-stage disease is abysmal with a median survival of <15 months. This case report describes a 38-year-old patient who presented with rapidly worsening abdominal distension. Subsequent debulking surgery removed a mass weighing 2.4kg, confirmed by histopathology as a high grade mucinous ovarian carcinoma with a mural nodule of anaplastic carcinoma. Evidence behind the current guidelines for management will be discussed, addressing our recent understanding of mOC as a separate disease from other histotypes and the consequent challenges in interpreting data from large multicentre trials in which patients with mOC are poorly represented. Moreover, using the Sister Mary Joseph nodule (SMJN) as an example, this case also highlights the importance of the physical examination and the value of subtle (and sometimes missed) clinical signs that provide important clues about the extent of a patient’s underlying disease and prognosis.

SWI/SNF-deficient malignancies of the female genital tract

Mutations and other molecular events involving subunits of the SWI/SNF chromatin remodelling complex are common in a wide variety of malignancies, including those arising at various sites in the female genital tract. Endometrioid and clear cell carcinomas in the uterine corpus and ovary not uncommonly contain mutations in ARID1A and these also occur in other endometriosis-associated ovarian neoplasms such as seromucinous tumours. In these organs, mutations in SMARCA4, SMARCB1, ARID1A and ARID1B (with subsequent loss of corresponding protein expression as a reliable surrogate) are relatively common in undifferentiated carcinomas, including the undifferentiated component of dedifferentiated carcinoma. SMARCA4 mutations are extremely common (almost ubiquitous) in small cell carcinoma of the ovary of hypercalcaemic type (SCCOHT), occurring in about 98% of these neoplasms, often in association with epigenetic SMARCA2 loss. SMARCB1-deficient vulval neoplasms include epithelioid sarcoma and myoepithelial carcinoma, as well as related malignancies which defy easy classification. Recently the spectrum of SWI/SNF deficient female genital malignancies has been expanded to include SMARCA4-deficient undifferentiated uterine sarcoma and mural nodules of anaplastic carcinoma in ovarian mucinous neoplasms.

Mural nodule of Anaplastic Carcinoma in an Ovarian Mucinous Cystadenoma- A rare case report

Mucinous tumors account approximately for 15% of all primary ovarian epithelial neoplasms. Ovarian mucinous tumors with mural nodules are rare surface epithelial-stromal tumorswhich can be of three types, “sarcoma-like”, sarcoma or anaplastic carcinoma. Mural nodules of anaplastic carcinoma were first described in 1982 by Prat et al following which approximately fifty cases have been reported to date and in those cases, only one has been associated with benign mucinous tumors. The current case report is from a 50-year-oldpostmenopausal woman who presented to the gynecology outpatient department with complaints of abdominal pain and distension for the past five months. On histopathological examination, a diagnosis of Mucinous cystadenoma with focal atypia and mural nodule of anaplastic carcinoma was made and confirmed with immunohistochemistry.

Ovarian mucinous tumors with mural nodules: immunohistochemical and molecular analysis of 3 cases

BackgroundPrimary ovarian mucinous tumors with mural nodules are very rare. The histogenesis of the mural nodules remains unclear.MethodsWe investigated the clincopathological and molecular features in 3 cases with mural nodules.ResultsPatient 1 was diagnosed as mucinous carcinoma with mural nodules of anaplastic carcinoma that was composed of CK+ and CK7+ spindled cells and polygonal cells with marked pleomorphism. Aberrant p53 staining was found in the mural nodules rather than in the mucinous components. A concordant KRAS mutation (c.35G > A p.G12A) was identified in both mucinous tumors and mural nodules. She died of disease at 44 months. The mural nodule in patient 2 was interpreted as a sarcoma, no other specified. The uniform short spindle cells were separated by abundant myxoid matrix. They were CD10 + , CCND1-, SMA-, and negative for break-apart BCOR, PHF1, and JAZF1 FISH assay. The adenocarcinomatous component harbored LOH at D18S51 and FGA loci while the sarcomatous component had LOH at D19S433. She had lung metastasis at 18 months and was alive without evidence of disease for 40 months. Patient 3 harbored multiple mural nodules that were composed of vimentin+, focal CK+, atypical spindle cells. A diagnosis of sarcoma-like mural nodules was rendered. She was alive with no evidence of disease for 13 months. No hotspot mutant AKT1, KRAS, HRAS, and PI3KCA alleles were found in patients 2 and 3.ConclusionsMural nodules with anaplastic carcinoma or with true sarcomas may represent the dedifferentiation form of mucinous tumors or collision tumors, respectively. The worrisome histology in sarcoma-like mural nodules necessitates meticulous treatment for these patients.

Ovarian Mucinous Cystadenoma with Anaplastic Carcinoma Arising in The Mural Nodule

Mural nodules in cystic ovarian neoplasms are very rare. Mural nodules are localized thickened nodular areas occurring in the wall of cystic mucinous or serous tumors of the ovary. Histologically they may be benign reactive sarcoma like lesions or malignant lesions like sarcomas and anaplastic carcinomas. We report a case of mural nodule of anaplastic carcinoma in a mucinous cystadenoma of ovary.

Molecular profiling study of a recurrent ovarian mucinous tumour with a mural nodule of anaplastic carcinoma, providing supportive evidence of dedifferentiation

Molecular profiling study of a recurrent ovarian mucinous tumour with a mural nodule of anaplastic carcinoma, providing supportive evidence of dedifferentiation

Ovarian Mucinous Tumor With Malignant Mural Nodules

Ovarian mucinous tumors with mural nodules are rare surface epithelial-stromal tumors. The mural nodules are divergent neoplasms that may be benign or malignant. The latter may be in the form of a sarcoma, carcinosarcoma, anaplastic carcinoma, or a variety of other recognized histotypes of carcinoma, which raises the question of whether malignant mural nodules represent a form of dedifferentiation in ovarian mucinous tumors or whether they represent collision tumors. We recently reported the K-RAS gene mutation status in a case of ovarian mucinous adenocarcinoma with mural nodule of high-grade sarcoma. The mucinous and sarcomatous components revealed a mutation in codon 12 of the K-RAS gene of a different nucleotide substitution, indicating that these 2 tumor components were different clones of the same tumor. Herein, we are reporting another case of a 20-yr-old woman who presented with 22 cm pelvic mass, omental caking, and ascites. A diagnosis of invasive mucinous carcinoma with mural nodules of anaplastic carcinoma was rendered. K-RAS gene mutation studies revealed p.G12V, c.35G>T mutation in the 2 components of the tumor, which is the most common mutation reported in mucinous tumors of the ovary. The fact that sarcomatous or anaplastic carcinomatous mural nodules in ovarian mucinous tumors display the same K-RAS mutations as their underlying mucinous neoplasms provides supportive evidence that at least some malignant mural nodules represent a form of dedifferentiation in ovarian mucinous tumors, rather than a collision of 2 divergent tumor types.

Une tumeur ovarienne mucineuse borderline avec des nodules muraux mixtes

The occurrence of mural nodules in serous or mucinous ovarian tumours is not frequent. Mural nodule can be developed in benign, borderline or malignant tumours. They can be benign, malignant or mixed type. Thus the prognosis of the ovarian tumour can be dramatically modified by the presence if these nodules. Eighty-two cases of mural nodules were reported in the literature, among which we account four cases of mixed nodules type. We report an additional case of mixed type mural nodules of anaplastic carcinoma and sarcoma-like developed in an ovarian mucinous borderline tumour at a 60-year-old woman.We give details about the classification, the differential diagnosis and prognosis of theses nodules.

Carcinosarcoma-like mural nodule in intestinal-type mucinous ovarian of borderline malignancy: a case report

Epithelial ovarian tumors of borderline malignancy are tumors with histologic features and biologic behavior between benign and frankly malignant epithelial ovarian neoplasms. To date, we cannot accurately predict the patients who are prone to an aggressive course of disease. Here, we present a 35-year-old patient with carcinosarcoma-like mural nodule in intestinal-type mucinous ovarian tumor of borderline malignancy. Foci of intraepithelial carcinoma (about 10%) without stromal invasion are also noted. Total hysterectomy, bilateral salpingo-oophorectomy, appendectomy, and omentectomy were performed, and the frozen pathology during operation showed mucinous tumor of borderline malignancy of left ovary on April 18, 2002. The patient was followed at our outpatient department for 19 months after operation and was free of the disease without any adjuvant chemotherapy. It is difficult to determine whether intestinal-type borderline mucinous tumors with intraepithelial carcinoma are associated with a worse prognosis compared with those with epithelial atypia alone due to disparate results in the published literature. In contrast, most patients with mural nodules of anaplastic carcinoma have had a malignant, often rapid, course. However, too few cases of carcinosarcoma-like mural nodule in mucinous tumor have been published to warrant a conclusion regarding their prognosis.

Carcinosarcoma-like mural nodule in intestinal-type mucinous ovarian of borderline malignancy: a case report

Epithelial ovarian tumors of borderline malignancy are tumors with histologic features and biologic behavior between benign and frankly malignant epithelial ovarian neoplasms. To date, we cannot accurately predict the patients who are prone to an aggressive course of disease. Here, we present a 35-year-old patient with carcinosarcoma-like mural nodule in intestinal-type mucinous ovarian tumor of borderline malignancy. Foci of intraepithelial carcinoma (about 10%) without stromal invasion are also noted. Total hysterectomy, bilateral salpingo-oophorectomy, appendectomy, and omentectomy were performed, and the frozen pathology during operation showed mucinous tumor of borderline malignancy of left ovary on April 18, 2002. The patient was followed at our outpatient department for 19 months after operation and was free of the disease without any adjuvant chemotherapy. It is difficult to determine whether intestinal-type borderline mucinous tumors with intraepithelial carcinoma are associated with a worse prognosis compared with those with epithelial atypia alone due to disparate results in the published literature. In contrast, most patients with mural nodules of anaplastic carcinoma have had a malignant, often rapid, course. However, too few cases of carcinosarcoma-like mural nodule in mucinous tumor have been published to warrant a conclusion regarding their prognosis.

Ductal Adenocarcinoma of the Pancreas with Huge Cystic Degeneration: A Lesion to Be Distinguished from Pseudocyst and Mucinous Cystadenocarcinoma

Cystic neoplasms of the pancreas are rare and often mistaken for pseudocyst by imaging studies and macroscopic examination. We describe an unusual tumor of the pancreas composed of a mural nodule of anaplastic carcinoma arising from a huge ductal adenocarcinoma undergoing cystic degeneration. The cyst measured 27 x 13 x 4 cm. Light microscopy showed that the cyst was partly lined by a single layer of cuboidal to columnar tumor cells with focal mucin production and was surrounded by hyalinized connective tissue. Most lining epithelial cells were absent owing to extensive degenerative process. Immunohistochemical studies showed positive staining of cytokeratin and vimentin for pleomorphic giant tumor cells, which were negative for leukocyte common antigen (CD45), KP-1 (CD68), epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA). The ductal adenocarcinoma stained strongly positive for cytokeratin and EMA, and negative for vimentin, CD45, CD68, and CEA. The clinical course of the current case was extremely poor and the prognosis resembled that of an anaplastic carcinoma. Therefore, we like to emphasize the importance of complete excision and extensive sampling of any cystic neoplasms in the pancreas including those with large cystic component to avoid missing the malignant elements.

A Case of Ovarian Mucinous Cystadenocarcinoma with Mural Nodule of Anaplastic Carcinoma

A Case of Ovarian Mucinous Cystadenocarcinoma with Mural Nodule of Anaplastic Carcinoma

Mural Nodules in Common Epithelial Tumors, of the Ovary

The occurrence of mural nodules in cystic common epithelial tumors of the ovary is well established, but differences in terminology and difficulties in histopathologic interpretation have hampered adequate understanding of their differential diagnoses and prognoses. Using immunohistochemistry and ultrastructural analyses supplementally, we studied two cases, one a serous cystadenocarcinoma with nodules of undifferentiated sarcoma, the other a mucinous cystadenocarcinoma with mural nodules of anaplastic carcinoma. Based on these cases and a review of 48 cases in the literature, we propose a standardized terminology. Mural nodules may be either reactive or neoplastic. Neoplastic mural nodules may be composed of benign elements, carcinoma, carcinoma with reactive elements, sarcoma, or an admixture of carcinoma and sarcoma; the latter category has some similarities to malignant mixed mesodermal tumors. The prognosis of patients with malignant mural nodules is poor, with 50% mortality. Strict morphologic criteria supplemented by immunohistochemistry aids in the sometimes difficult differential diagnosis among these types of mural nodules.

Ovarian Mucinous Cystadenocarcinoma with Sarcoma-Appearing Mural Nodule of Anaplastic Carcinoma

An ovarian mucinous cystadenocarcinoma with sarcoma-appearing solid mural nodules in an 18-year-old Japanese woman is reported. Right ovarian cyst had two poorly circumscribed, solid nodules and several tiny studs. Microscopically, the epithelial elements consisted of benign, low malignant potential and malignant mucinous tumors. The mural nodules were made up of highly malignant anaplastic cells simulating sarcoma. In one of the mural nodules, continuity between the malignant mucinous cells and sarcoma-appearing cells was identified. Immunohistochemically, the sarcoma-appearing nodule was uniformly positive for vimentin and focally positive for epithelial markers such as EMA, CAM5.2, and AE1/AE3. This case demonstrated an orderly transformation and dedifferentiation of epithelial cells to undifferentiated mesenchymal cells. Although she had stage IIb disease, she lived disease free for 3 years and 5 months after surgery followed by 10 courses of chemotherapy containing cisplatin and Adriamycin.

Ovarian mucinous tumor with mural nodules of anaplastic carcinoma

A case of mucinous cystic ovarian tumor with mural nodules of anaplastic carcinoma in a 30-year-old woman is described. The carcinomatous components within the nodules showed strong immunopositivity for cytokeratin and carcinoembryonic antigen, and ultrastructurally they displayed epithelial and glandular differentiation. Omental metastasis had already developed in the patient, and she received postoperative adjuvant chemotherapy consisting of cyclophosphamide and cis-platinum. No sign of recurrence was evident 4 months after the operation. The literature is reviewed and the importance of adjuvant chemotherapy in the postoperative management of such patients highlighted. The salient pathologic features differentiating mural nodules of anaplastic carcinoma and true sarcoma from prognostically favorable sarcoma-like nodules are presented.