scholarly journals Mucinous appendiceal adenocarcinomas: a diagnostic challenge

2020 ◽  
Vol 2020 (1) ◽  
Author(s):  
Ekta Sharma ◽  
Shyam Ajay Gokani ◽  
Jonathan Neville ◽  
Ashish Sinha ◽  
Tushar Agarwal
Keyword(s):  
Diagnostic Laparoscopy ◽  
District General Hospital ◽  
Low Grade ◽  
High Grade ◽  
Diagnostic Challenge ◽  
Mucinous Neoplasm ◽  
Post Surgery ◽  
Appendiceal Neoplasms ◽  
Specific Symptoms ◽  
Radiological Studies

Abstract Mucinous appendiceal neoplasms are rare and diagnosis is often difficult as patients present with non-specific symptoms often suggestive of appendicitis only and most diagnoses are made following post-surgery histology examination. Here we present a case of a mucinous appendiceal adenocarcinoma with areas of both Low-grade Appendiceal Mucinous Neoplasm and High-grade Appendiceal Mucinous Neoplasm, in a ruptured appendix at a district general hospital. Without early detection and treatment these pathologies can lead to pseudomyxoma peritonei, a syndrome of progressive mucinous ascites. This case highlights the limitations of radiological studies, benefits of diagnostic laparoscopy and provides a rationale for removing the mesoappendix with the appendix during routine appendectomies.

scholarly journals Classification of mucinous appendiceal neoplasm and pseudomyoxma peritonei

2018 ◽  
Vol 8 (1) ◽  
pp. 1301-1307
Author(s):  
Arnab Ghosh
Keyword(s):  
Goblet Cell ◽  
Wide Spectrum ◽  
Low Grade ◽  
Controversial Issues ◽  
High Grade ◽  
Goblet Cell Carcinoid ◽  
Signet Ring Cells ◽  
Mucinous Neoplasm ◽  
Signet Ring

Mucinous appendiceal tumors are uncommon and include a wide spectrum of tumors whose classification remained controversial. Some of these mucin producing appendiceal tumors can disseminate to the peritoneal cavity leading to pseudomyxoma peritonei (PMP). Despite several attempts to classify mucinous tumors of appendix and PMP by different authors in the past, no universally accepted classification system was present. The controversial issues were discussed at the 2012 World Congress of the Peritoneal Surface Oncology Group International (PSOGI) in Berlin. A panel of 71 experts from 13 different countries was formed under the lead co-ordinator Norman J. Carr. A total of 4 rounds of questionnaires and one meeting were held. The opinion of the majority was taken into account. Importance of intactness of muscularis mucosae, pushing invasion and infiltrative invasion were emphasized. The entities Low grade appendiceal mucinous neoplasm (LAMN) and High grade appendiceal mucinous neoplasm (HAMN) were defined.. The terminologies suggested for Goblet cell carcinoid and adenoneuroendocrine carcinoma were goblet cell tumor and adenocarcinoma ex goblet cell carcinoid. Acellular mucin in peritoneum was not classified under PMP which was classified into 3 categories depending upon low grade , high grade cytologic features and presence of signet ring cells. It was suggested to report the extent of mucin and cells separately. A reporting format solely for mucinous appendiceal tumors was formulated by the panel. However, there are some grey areas which may have to be addressed in future.

Is there a role for neoadjuvant chemotherapy in the management of non-carcinoid epithelial neoplasms of the appendix?

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 15115-15115
Author(s):  
M. H. Katz ◽  
P. F. Mansfield ◽  
C. Eng ◽  
R. A. Wolff ◽  
P. Diaz ◽  
...  
Keyword(s):  
Neoadjuvant Chemotherapy ◽  
Standard Of Care ◽  
Low Grade ◽  
High Grade ◽  
Appendiceal Neoplasms ◽  
Tertiary Center ◽  
Standardized Treatment ◽  
Crs And Hipec ◽  
Epithelial Neoplasms

15115 Background: Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS and HIPEC) are considered the standard of care for patients with pseudomyxoma peritonei (PMP) and carcinomatosis of appendiceal origin. The role of neoadjuvant chemotherapy (CTX) in the management of these patients is unknown. Methods: Retrospective analysis of all patients evaluated for the treatment of appendiceal epithelial neoplasms at a tertiary center between 1992 and August 2006. All diagnoses were confirmed pathologically and classified by a single group of pathologists. Patients with carcinoid tumors or metastases to the appendix were excluded. Tumor histology, stage, peritoneal-based disease, and the potential for complete cytoreduction dictated treatment. Results: 250 consecutive patients were evaluated, 140 of whom had low grade disease. 114 underwent CRS and HIPEC. Median follow-up was 24 mos from referral. Average time to referral was 13 mos after diagnosis (range 0–243); 85% had undergone prior surgical therapy (median 1.3 operations, range 1–4); 22% had previous CTX consisting of many different regimens. 5- and 10-year survival for patients with low grade tumors treated with CRS and HIPEC alone (n=80) were 84% and 68%, respectively. 21 patients with low grade tumors had CTX prior to CRS and HIPEC. There was no effect on overall survival (p = 0.61). 5-and 10-year survival of 39 patients with low grade histology who did not receive CRS and HIPEC was 55% and 30%, respectively (p = 0.009). 83 patients with intermediate and high-grade disease who received CTX but not CRS and HIPEC had a 5- year survival of 27%. 5-year survival for patients with intermediate or high grade disease who underwent CRS and HIPEC (n=13) was 67%. Conclusions: Patients with peritoneal-based disease from non-carcinoid epithelial neoplasms of the appendix who undergo CRS and HIPEC have a more favorable survival. Currently there is no survival advantage to the use of CTX before CRS and HIPEC for low grade appendiceal neoplasms. The role of neoadjuvant CTX and biologic agents for patients with high grade neoplasms needs to be determined. Early referral to a peritoneal malignancy center will help standardized treatment for these patients. No significant financial relationships to disclose.

scholarly journals High Grade Serous Carcinoma Presenting as a Huge Pelvic Mass with Normal Looking Ovaries: A Diagnostic Challenge for Pathologist

2020 ◽  
Author(s):  
Vaishali Walke ◽  
Amrapali Gaikwad ◽  
Madiha Shaikh ◽  
Balwant Kowe
Keyword(s):  
Correct Diagnosis ◽  
Diagnostic Delay ◽  
Delayed Diagnosis ◽  
Pelvic Mass ◽  
Clinical Findings ◽  
Serous Carcinoma ◽  
High Grade ◽  
Diagnostic Challenge ◽  
Immunohistochemical Markers ◽  
Specific Symptoms

High-grade serous carcinoma represents 50-60% of all ovarian cancers and is the most common type of malignant surface epithelial tumour. Serous carcinoma is often diagnosed in the sixth and seventh decade, while the mean age for high-grade tumours is 63 year. It’s not only the non-specific symptoms like vague abdominal pain, nausea, gastrointestinal disturbances, but also the unusual presentation which is responsible for delayed diagnosis. This diagnostic delay can become the reason for increased mortality, despite advances in surgical management and chemotherapy. Here, authors discuss a case of 60-year-old postmenopausal women who presented with a huge pelvi-peritoneal mass and with grossly normal ovaries. Considering clinical findings and histomorphology, the possibility of malignant mesothelioma was considered as the first differential diagnosis; however extensive sampling of ovaries and supportive immunohistochemical markers helped us to arrive at a definitive diagnosis. The present case emphasises the importance of extensive tissue sampling and ancillary techniques in arriving at a correct diagnosis.

scholarly journals Transformation of Low-Grade Mucinous Neoplasm of the Appendix With Pseudomyxoma Peritonei to High-Grade Sarcomatoid Carcinoma

2015 ◽  
Vol 7 (7) ◽  
pp. 571-574 ◽  
Author(s):  
Yu-Nung Chen ◽  
Jie-Jen Lee ◽  
Shih-Ping Cheng ◽  
Chung-Hsin Tsai
Keyword(s):  
Pseudomyxoma Peritonei ◽  
Sarcomatoid Carcinoma ◽  
Low Grade ◽  
High Grade ◽  
Mucinous Neoplasm

Clinicopathological Characteristics of Low-Grade Appendiceal Mucinous Neoplasm

2021 ◽  
pp. 1-8
Author(s):  
Tatsuro Yamaguchi ◽  
Kohei Murata ◽  
Tetsuya Shiota ◽  
Hiroshi Takeyama ◽  
Shingo Noura ◽  
...  
Keyword(s):  
Distant Metastasis ◽  
Clinicopathological Characteristics ◽  
Complete Resection ◽  
Low Grade ◽  
Mucinous Neoplasm ◽  
Node Metastasis ◽  
Appendiceal Neoplasms ◽  
Mucinous Neoplasms ◽  
Mucinous Epithelium ◽  
The Difference

Introduction: Recently, “low-grade appendiceal mucinous neoplasms” (LAMNs) have been proposed as one subtype of appendiceal mucinous neoplasms, characterized by a villous or flat proliferation of mucinous epithelium with low-grade cytologic atypia. The aim of this study was to clarify the clinicopathological characteristics of LAMN. Methods: In this multi-institutional cohort study, we retrospectively analyzed the clinicopathological characteristics in appendiceal neoplasms patients who underwent treatment from 2000 to 2017. Results: In total, 922 patients were enrolled, with 279 (30.3%) cases of LAMN, and 93 (10.1%) cases of non-LAMN disease. In comparison with patients with non-LAMN disease, those with LAMN had significantly lower levels of CA19-9 (p = 0.045), a lower frequency of T4 tumors (p < 0.0001), a lower frequency of lymph node metastasis (p < 0.0001), and a lower frequency of distant metastasis (p < 0.0001). Survival analysis revealed that patients with LAMN had a significantly better prognosis than did those with non-LAMN disease (p < 0.001). Among the patients with distant metastasis, those with LAMN had a significantly better prognosis than did those with non-LAMN disease (p = 0.0020), but among the patients without distant metastasis, the difference between the 2 groups was not significant (p = 0.26). However, among patients who underwent complete resection, the difference in prognosis between the 2 groups was not significant (p = 0.10). Conclusions: A multicenter retrospective study revealed that the clinicopathological characteristics of LAMN was different from those of non-LAMN.

Aromatase inhibitor maintenance therapy in high grade advanced ovarian cancer to delay first recurrence.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 5515-5515 ◽  
Author(s):  
Alexandra Maria Knipprath-Mészáros ◽  
Marcus Vetter ◽  
Celine Montavon ◽  
Francesco Vigo ◽  
Andreas Schoetzau ◽  
...  
Keyword(s):  
Ovarian Cancer ◽  
Maintenance Therapy ◽  
Residual Disease ◽  
Control Group ◽  
Low Grade ◽  
High Grade ◽  
Advanced Stage ◽  
Post Surgery ◽  
Free Interval ◽  
Antihormonal Treatment

5515 Background: Ovarian cancer (OC) is mostly diagnosed as G3 serous advanced stage disease with survival of only 20% in 5 years. Maintenance therapy after 1st line chemotherapy is increasingly used, particularly with Bevacizumab or PARP inhibitors. However, the effect of antihormonal treatment in breast cancer but also in relapsed gynecological cancers has been shown. Also in low grade OC, data are highly supportive of antihormonal treatment. In a previous study of ours, also high grade serous cancers express high amounts of estrogen receptor (ER). The aim of this study was to analyze whether a maintenance antihormonal therapy in advanced OC adds a benefit in relation to the time of recurrence. Methods: All newly diagnosed G3 FIGO III/IV OC cases at our Hospital were assessed prospectively for ER expression. Patients with positive ER status (> 10%) were treated as maintenance therapy with Letrozol 2.5mg 1x/d or not. Progression free survival was recorded and analyzed according to Kaplan-Meier. Patients with macroscopic residual disease post surgery receiving Bevacizumab maintenance treatment were also included. Results: We identified 51 patients with G3 serous OC FIGO III/IV expressing ER. Hereby, 24 patients received and 27 patients did not receive Letrozol after adjuvant chemotherapy. Time to progression ranged from 4 to 121 months. The use of Letrozol was associated with a significant prolonged progression free interval. After 12 months, only 65% of women in the control group vs 84% in the Letrozol group were progression-free. After 24 months, the effect was even stronger (46% in the control group vs 74% with Letrozol (p= 0.02)). Within the subgroup of patients with residual disease treated with Bevacizumab a similar effect was seen with 41% of patients progression free after 12 months vs 89% when taking Letrozol in addition to Bevacizumab. Conclusions: The use of Letrozol as a maintenance therapy after the 1st line treatment in G3 advanced stage serous OC patients was associated with a longer recurrence free interval in our cohort. These findings warrant a randomized controlled trial comparing all existing maintenance regimen as this might have a major influence on cost development in OC treatment.

scholarly journals Vulvar Paget disease secondary to high-grade urothelial carcinoma with underlying massive vascular embolization and cervical involvement: case report of unusual presentation

2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Walquiria Quida Salles Pereira Primo ◽  
Guttenberg Rodrigues Pereira Primo ◽  
Dunya Bachour Basilio ◽  
Karime Kalil Machado ◽  
Jesus Paula Carvalho ◽  
...  
Keyword(s):  
Urothelial Carcinoma ◽  
Immunohistochemical Analysis ◽  
Low Grade ◽  
High Grade ◽  
Paget Disease ◽  
Careful Clinical Examination ◽  
History Of ◽  
Carcinoma Of The Bladder ◽  
Tumor Emboli ◽  
Specific Symptoms

Abstract Background Vulvar extramammary Paget disease is a rare chronic condition, that presents with non-specific symptoms such as pruritus and eczematous lesions. Because most of these lesions are noninvasive, the distinction between primary and secondary Paget disease is crucial to management. Case presentation We report an unusual case of vulvar Paget disease associated with massive dermal vascular embolization, cervicovaginal involvement and metastasis to inguinal and retroperitoneal lymph nodes. The intraepithelial vulvar lesion had a classical appearance and was accompanied by extensive component of dermal lymphovascular tumor emboli, similar to those observed in inflammatory breast carcinoma. Immunohistochemical analysis revealed that the lesion was secondary to high-grade urothelial cell carcinoma. The patient had a history of superficial low-grade papillary urothelial carcinoma of the bladder, which had appeared 2 years before the onset of vulvar symptoms. Conclusions Eczematoid vulvar lesions merit careful clinical examination and biopsy, including vulva mapping and immunohistochemistry. The information obtained may help to define and classify a particular presentation of Paget disease. Noninvasive primary lesions do not require the same aggressive approaches required for the treatment of invasive and secondary disease.

scholarly journals Ovarian Serous Carcinoma Associated With a Distinct “Corded and Hyalinized” Pattern

2013 ◽  
Vol 137 (2) ◽  
pp. 275-279 ◽  
Author(s):  
Jeannelyn S. Estrella ◽  
Judith K. Wolf ◽  
Michael T. Deavers
Keyword(s):  
Distinct Pattern ◽  
Serous Carcinoma ◽  
Second Primary ◽  
Low Grade ◽  
High Grade ◽  
Diagnostic Challenge ◽  
Unique Case ◽  
Spindle Cell Neoplasm ◽  
High Grade Carcinoma ◽  
Collagenous Stroma

The “corded and hyalinized” pattern, described in endometrioid carcinoma, has not been previously reported in association with serous carcinoma. We describe a unique case of serous neoplasm of low malignant potential with low-grade serous carcinoma combined with a distinct pattern of high-grade carcinoma characterized by cords of epithelioid and spindled cells enmeshed in a hyalinized, collagenous stroma. This pattern was the predominant architecture in the patient's recurrence and caused a diagnostic challenge, as the splenic recurrence was initially diagnosed as a second primary high-grade spindle cell neoplasm. Both ovarian and splenic tumors displayed positive immunohistochemical staining for cytokeratin 7, cytokeratin 8/18, estrogen receptor, and paired box gene 8 (PAX-8) in the conventional serous carcinoma and the corded and hyalinized component, confirming the diagnosis of recurrent carcinoma. The behavior in this unique case of serous carcinoma associated with a distinct corded and hyalinized pattern was more aggressive than low-grade serous carcinoma, but more favorable than malignant mixed mullerian tumor.

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