Mucinous appendiceal neoplasms: pathologic classification, clinical implications, imaging spectrum and mimics

Hooser, Alanna Van; Williams, Todd R.; Myers, Daniel T.

doi:10.1007/s00261-018-1561-9

Cited by 37 publications

(51 citation statements)

References 17 publications

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“…This study has highlighted a notable difference in the ‘macroscopic’ demonstration of complicated ADD with mucus cysts and LAMNs. The latter are often very obvious, both radiologically and at the time of surgery . In contrast, in this series only in eight cases (11%) were the macroscopic features noted at the time of surgery to be suggestive of any pathology in addition to acute appendicitis.…”

Section: Discussioncontrasting

confidence: 66%

“…The latter are often very obvious, both radiologically and at the time of surgery. 19 In contrast, in this series only in eight cases (11%) were the macroscopic features noted at the time of surgery to be suggestive of any pathology in addition to acute appendicitis. This relative lack of surgical or often macroscopic pathological suspicion of a mucin-producing neoplasm should serve as a diagnostic aid to the pathologist to favour complicated ADD, rather than LAMN.…”

Section: Discussioncontrasting

confidence: 61%

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Complicated appendiceal diverticulosis versus low‐grade appendiceal mucinous neoplasms: a major diagnostic dilemma

et al. 2019

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Aims To research and identify how often complicated diverticular disease of the appendix [appendiceal diverticular disease (ADD)] shows histological mimicry of low‐grade appendiceal mucinous neoplasms (LAMNs) and to provide guidance on the useful histopathological features that allow the appropriate diagnosis to be made. Methods and results Seventy‐four cases of complicated appendiceal diverticular disease were identified from two specialist centres. Of the second opinion/consultation cases, 71% of the ADD cases had been diagnosed by referring pathologists as LAMNs. Salient pathological features were identified and agreed upon to reach the applicable diagnosis. For a diagnosis of complicated diverticulosis, particularly when associated with mucus cysts, the following morphological features were regarded as important: relative retention of the normal mucosal architecture with lamina propria and a maintained crypt architecture, crypts arranged in regular array, epithelial hyperplasia and a lack of nuclear abnormalities extending the length of the crypts. In a formal case–control study undertaken on 30 cases with each diagnosis, ADD and LAMN, loss of lamina propria, a filiform architecture and hypermucinosis were significantly associated with low‐grade appendiceal mucinous neoplasms. Mucosal neuromas were significantly associated with diverticular disease of the appendix. Conclusions To our knowledge, this study represents the largest series in the world literature and serves to highlight the important pathological features to distinguish complicated diverticular disease of the appendix from LAMNs, and emphasises the difficulties experienced by diagnostic pathologists in diagnosing complicated appendiceal diverticulosis. This is important, as LAMNs have a significant risk of transcoelomic spread, while complicated appendiceal diverticulosis has no such risk.

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Section: Discussioncontrasting

confidence: 66%

Section: Discussioncontrasting

confidence: 61%

Complicated appendiceal diverticulosis versus low‐grade appendiceal mucinous neoplasms: a major diagnostic dilemma

et al. 2019

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“…PMP is a spread of gelatinous material (mucin) from the lumen of the appendix into the abdominal cavity, morphologically referred to as mucoceles [5]. The median time to PMP development after MAA onset is~2 years [6]. With peritoneal metastasis, the five-year survival rate for mucinous adenocarcinoma is reported as low as 25% [6].…”

Section: Introductionmentioning

confidence: 99%

“…The median time to PMP development after MAA onset is~2 years [6]. With peritoneal metastasis, the five-year survival rate for mucinous adenocarcinoma is reported as low as 25% [6]. In addition, mucinous adenocarcinomas have a relatively higher risk for hematogenous and nodal metastasis [6].…”

Section: Introductionmentioning

confidence: 99%

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Late stage diagnosis of mucinous adenocarcinoma of the appendix: a case report of an unusual tumor with a rare presentation

et al. 2020

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Background: The incidence of mucinous appendiceal adenocarcinomas (MAA) has increased over the past three decades. Advanced stage tumor diagnosis is likely attributable to non-specific findings. Here we describe advanced stage appendiceal MAA presenting as inguinal ulcers, scrotal abscesses, and other nonspecific symptoms. To our knowledge, this is the first report of MAA presenting as inguinal pain with inflamed phlegmonous tissue and scrotal abscess. Case presentation: A 67-year-old male presented to a rural facility complaining of weight-loss, fatigue, hematuria, dysuria, painful right inguinal ulceration, and right scrotal abscess drainage. Computed tomography of the abdomen and pelvis revealed a distended appendix (> 1.3 cm) and a fistula between the appendix, urinary bladder, right scrotum, and right groin. Laparoscopic appendectomy was performed and diagnosed as MAA. After a right hemicolectomy, the MAA was staged as pT3b pN0 M0 G2. Conclusion: This case highlights a unique presentation of late stage appendiceal MAA. Due to the increased incidence of appendiceal MAAs, reports of unique clinical features are needed to facilitate early diagnosis and intervention, especially in rural settings with limited access to specialists.

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The ‘onion skin’ sign of a low‐grade appendiceal mucinous neoplasm: An incidental finding during early pregnancy assessment

Fourie,

Al Memar,

Tuomey

et al. 2024

Australas J Ultrason Med

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A low‐grade appendiceal mucinous neoplasm (LAMN) is a cystic dilatation of the appendix resulting from the accumulation of mucinous secretions caused by a luminal obstruction. Although usually benign, pseudomyxoma peritonei may occur in the event of rupture, and 10% of cases may be secondary to appendiceal cystadenocarcinoma. A LAMN is both more common and more likely to have a malignant association in women, making it an entity with which practitioners of gynaecological ultrasound should be familiar. Although not the primary aim, early pregnancy ultrasound assessments can offer the diagnostic opportunity to identify pelvic pathology. A LAMN can be identified on ultrasonography by visualisation of an adnexal mass separate to the ovary, which due to the layers of secretions has a distinctive appearance previously likened to ‘onion‐skin’ or ‘whipped‐cream’. Here, we describe an incidental finding of a LAMN during an early pregnancy assessment. Practitioners of early pregnancy ultrasound should be familiar with the characteristic morphology of this rare but important finding.

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Mucinous appendiceal neoplasms: pathologic classification, clinical implications, imaging spectrum and mimics

Cited by 37 publications

References 17 publications

Complicated appendiceal diverticulosis versus low‐grade appendiceal mucinous neoplasms: a major diagnostic dilemma

Complicated appendiceal diverticulosis versus low‐grade appendiceal mucinous neoplasms: a major diagnostic dilemma

Late stage diagnosis of mucinous adenocarcinoma of the appendix: a case report of an unusual tumor with a rare presentation

The ‘onion skin’ sign of a low‐grade appendiceal mucinous neoplasm: An incidental finding during early pregnancy assessment

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