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Erschienen in: Journal of Medical Case Reports 1/2022

Open Access 01.12.2022 | Case report

Adenomatoid mesothelioma arising from the diaphragm: a case report and review of the literature

verfasst von: Kenta Kawabe, Hiroki Sato, Akiko Kitano, Ryuichi Yoshida, Kazuya Yasui, Yuzo Umeda, Kazuhiro Yoshida, Tomokazu Fuji, Kenjiro Kumano, Kosei Takagi, Masaaki Kagoura, Takahito Yagi, Toshiyoshi Fujiwara

Erschienen in: Journal of Medical Case Reports | Ausgabe 1/2022

Abstract

Background

Adenomatoid mesothelioma is a rare subtype of malignant mesothelioma that can be confused with adenomatoid tumors, which are classified as benign. The clinical features and optimal management of adenomatoid mesothelioma have not been elucidated in the literature. In this report, we present an extremely rare case of adenomatoid mesothelioma that developed on the peritoneal surface of the diaphragm as well as a literature review of adenomatoid mesothelioma in the abdominal cavity.

Case presentation

The patient was a 61-year-old Japanese woman who had undergone resection of a malignant peripheral nerve sheath tumor of the hand 18 years prior. She was diagnosed with clinical stage I lung adenocarcinoma on follow-up chest radiography. Simultaneously, a 20-mm enhancing nodule with slow growth on the right diaphragm was detected on contrast-enhanced computed tomography. She presented no specific clinical symptoms. At this point, the lesion was suspected to be a hypervascular tumor of borderline malignancy, such as a solitary fibrous tumor. After a left upper lobectomy for lung adenocarcinoma, she was referred to our department, and laparoscopic tumor resection was performed. Adenomatoid tumors were also considered based on the histopathological and immunohistochemical analyses, but we made the final diagnosis of adenomatoid mesothelioma using the results of the genetic profile. The patient remains alive, with no recurrence noted 6 months after surgery.

Conclusion

We encountered a valuable case of adenomatoid mesothelioma of peritoneal origin. There are some previously reported cases of adenomatoid mesothelioma and adenomatoid tumors that may need to be recategorized according to the current classification. It is important to accumulate and share new findings to clarify the clinicopathological characteristics and genetic status of adenomatoid mesothelioma.
Hinweise

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Abkürzungen
CT
Computed tomography
US
Ultrasonography
SFT
Solitary fibrous tumor
FISH
Fluorescence in situ hybridization
CEA
Carcinoembryonic antigen
TTF-1
Thyroid transcription factor-1
CD34
Cluster of differentiation 34
STAT6
Signal transducer and activator of transcription 6
TRAF7
TNF receptor associated factor 7
CK 5/6
Cytokeratin 5/6

Background

Malignant mesothelioma is classified into three main histological subtypes as follows: epithelial, sarcomatoid, and biphasic. According to the recent World Health Organization classification of pleural tumors, adenomatoid mesothelioma is categorized as a rare subtype of epithelioid mesothelioma that should be distinguished from adenomatoid tumors, which are benign mesothelial tumors [1, 2]. It is reported that adenomatoid mesothelioma accounts for approximately 5% of pleural mesothelioma and can present various types of morphologic features, leading to difficulty in diagnosis [3]. Due to the rarity of reported cases, the clinical features and optimal management of adenomatoid mesothelioma have not been described yet. Herein, we report an extremely rare case of adenomatoid mesothelioma that developed on the peritoneal surface of the diaphragm as well as a literature review of adenomatoid mesothelioma in the abdominal cavity.

Case presentation

A 61-year-old Japanese woman underwent resection of a malignant peripheral nerve sheath tumor of the hand when she was aged 43 years and was followed up by radiological examination. Chest radiography revealed a mass lesion in the left upper lung 18 years later. She was a current smoker but had no history of asbestos exposure, and presented no specific clinical symptoms. As a result of a detailed examination, she was diagnosed with clinical stage I lung adenocarcinoma. Simultaneously, contrast-enhanced computed tomography (CT) detected a 20-mm enhancing nodule with slow growth on the right diaphragm (Fig. 1a). 18-Fluoro-2-deoxyglucose positron emission tomography revealed that the maximum standard uptake value of the nodule was 3.5 (Fig. 1b). Ultrasonography (US) revealed a low-echoic lesion, and early enhancement was observed on Sonazoid-enhanced US (Fig. 1c, d). These results indicated that the lesion was a hypervascular tumor of borderline malignancy, such as solitary fibrous tumor (SFT). After a left upper lobectomy for lung adenocarcinoma, the patient was referred to our department for surgical resection of the peritoneal tumor. Laboratory data at the time of presentation were as follows: white blood cell count, 7240 cells/μL; hemoglobin level, 14.2 g/dL; platelet count, 10.4 × 104 cells/μL; aspartate transaminase, 22 IU/L; alanine aminotransferase, 24 IU/L; total bilirubin, 0.57 mg/dL; albumin, 4.1 g/dL; creatinine, 0.58 mg/dL. Serum tumor markers, such as proteins induced by vitamin K absence or antagonist II and alpha-fetoprotein, were within the normal range (25 μg/mL and 2.1 ng/mL, respectively). Laparoscopic tumor resection was performed. Intraoperative findings are shown in Fig. 2. A thin pedunculated tumor was found to originate from the peritoneal surface of the right diaphragm. The tumor was compressing liver segment 8 but without apparent invasion. Well-developed capillary vessels were observed around the tumor. The pedicle of the tumor was clipped at its origin and divided, and a tumorectomy was completed. Gross examination showed a 28 × 20 x 11 mm3 brown–red tumor with a smooth cut surface (Fig. 3a). Histopathological examination revealed papillary architecture with focal small aggregates of mesothelial cells (Fig. 3b). Glandular lumen formation, indicative of an adenomatoid pattern, was partially observed. Immunohistochemical analysis showed that the tumor cells were positive for cytokeratin 5/6 (CK 5/6) and calretinin, and negative for carcinoembryonic antigen (CEA), thyroid transcription factor-1 (TTF-1), cluster of differentiation 34 (CD34), and signal transducer and activator of transcription 6 (STAT6) (Fig. 3c–h). In addition, hot-spot mutations in TNF receptor associated factor 7 (TRAF7) were not detected by Sanger sequencing, and the tumor cells displayed negative immunostaining for L1 cell adhesion molecule. Fluorescence in situ hybridization (FISH) showed no homozygous deletion of 9p21 or hemizygous deletion of NF2 (data not shown). Based on these results, the patient was diagnosed with localized adenomatoid mesothelioma. The postoperative course was uneventful, and the patient was discharged on the fourth postoperative day. She remains alive and is being monitored in an outpatient setting. No recurrence was noted 6 months after surgery.

Discussion and conclusions

Malignant mesothelioma is a highly aggressive tumor arising from mesothelial cells lining the pleura, peritoneum, and pericardium. The prognosis differs among histological subtypes, and epithelioid mesothelioma contains several morphological subtypes that also have some impact on prognosis [4, 5]. Adenomatoid mesothelioma is usually classified as an architectural subtype of epithelioid mesothelioma. It can histologically mimic adenomatoid tumors and is sometimes difficult to distinguish from others [3]. We report herein a case of peritoneal adenomatoid mesothelioma originating in the diaphragm. There are several reports of pleural adenomatoid mesothelioma, but adenomatoid mesothelioma of peritoneal origin is extremely rare. To the best of our knowledge, this is the first report of peritoneal adenomatoid mesothelioma localized to the diaphragm.
In the present patient, we made the final pathological diagnosis of localized adenomatoid mesothelioma based on histochemical and immunohistochemical analyses and genetic profiling. Based on the tumor growth rate and enhancement pattern on contrast-enhanced CT, we first suspected an SFT. However, immunohistochemical staining was negative for CD34 and STAT6, ruling out the possibility of SFTs. Metastasis of lung adenocarcinoma was also ruled out based on the immunohistochemical staining patterns for positive mesothelial and negative epithelial markers (positive for CK5/6 and calretinin and negative for CEA and TTF-1). The tumor was thought to be an adenomatoid tumor or adenomatoid mesothelioma. Several reports indicate that adenomatoid tumors are defined by TRAF7 mutation; therefore, we examined TRAF7 hot-spot mutations, including codons 519, 521, 538, 561, and 577, using Sanger sequencing [6, 7]. TRAF7 mutations were not detected. Homozygous deletion of 9p21 and hemizygous deletion of NF2, which are characteristics of pleural mesothelioma, was also not detected by FISH. However, the frequency of these genetic alterations in peritoneal mesothelioma is reported to be less than that of pleural mesothelioma, which led to the final diagnosis of adenomatoid mesothelioma [8]. In addition to a pathological examination, a genetic approach may be necessary and useful for distinguishing adenomatoid mesothelioma from adenomatoid tumors.
We conducted a literature search of the PubMed database to understand the current status of adenomatoid mesothelioma in the abdominal cavity. We came across six cases, including our case, that reported an “adenomatoid mesothelioma” (Table 1) [913]. There was one man and five women, and none had a history of asbestos exposure. The sites of tumor origin included the uterus (n = 1), testis (n = 1), liver (n = 1), and peritoneum (n = 4), and multiple tumors were observed in patient 5. Recurrence was not observed in five out of six patients, but Mori et al. reported a recurrent case after curative resection (patient 5) [13]. We should also conduct a careful follow-up with our patient, although the optimal follow-up strategy has not been well established. Through a literature search, we found that there were patients in whom it was difficult to distinguish between adenomatoid tumors and adenomatoid mesothelioma. For instance, several reports mentioned adenomatoid mesothelioma as a “benign tumor.” However, considering that adenomatoid mesothelioma is categorized as a subtype of epithelial mesothelioma in the latest World Health Organization classification, it should be treated as a malignant tumor. In addition, there are several reports of adenomatoid tumors invading the surrounding organs. Adenomatoid tumors are classified as benign; therefore, these patients may need to be recategorized according to the current classification. It is necessary to clearly define adenomatoid mesothelioma not only from the viewpoint of morphology but also from the viewpoint of mutational status, such as TRAF7 mutation and immunostaining findings, which would enable us to examine the clinical characteristics of adenomatoid mesothelioma.
Table 1
Reported cases of adenomatoid mesothelioma in the abdominal cavity
Case
Study
Age
Sex
Asbestos
Tumor origin
Note
Diameter
Clinical symptoms
Surgery
Pathological diagnosis
Recurrence
Later course
1
Bisset et al. 1988
45
F
Uterus
Approximately 10 cm, No recurrence for 3 years
10 cm
Heavy, frequent menstrual bleeding
Hysterectomy
Giant cystic adenomatoid tumor (mesothelioma)
No, 3 years
Remains well 3 years later
2
Lins et al. 2009
25
F
Peritoneum
Approximately 10 cm, No recurrence for 7 years
10.8 cm
Pelvic pain
Tumorectomy
Adenomatoid mesothelioma
No, 7 years
Free of the disease for 7 years
3
Okuda et al. 2014
61
F
Peritoneum
Invasion to ovary
UK
None
Laparoscopic right salpingo-oophorectomy and adhesiotomy
Adenomatoid-like mesothelioma
UK
Two months later, the patient underwent laparoscopic segmental resection of the sigmoid colon, with histological analysis identifying an adenomatoid-like tumor. No information about later course
4
Yang et al. 2014
68
M
Testis
Invasion to tunica vaginalis and spermatic cord
6.8 cm
Swelling and slightly painful in the right scrotum
Radical orchiectomy
Mesothelioma with prominent adenomatoid features
No
The clinical follow-up was done at 3 months and 6 months after the surgery, respectively. No recurrence or metastasis of the tumor was observed
5
Mori et al. 2020
60
F
Lliver surface, pelvic cavity and anterior peritoneum
Invasion to blood vessel, multiple recurrences (peritoneum, lung). Alive at 7 years after surgery
UK
None
Laparoscopic tumor resection
Adenomatoid (microcystic) mesothelioma
Yes
Relapsed 4 years later and metastasized to the lung, but the patient remains alive 7 years after the first tumor resection surgery, while receiving chemotherapy.
6
Our case
61
F
 
Peritoneum (diaphragm)
No invasion
2.8 cm
None
Laparoscopic tumor resection
Adenomatoid mesothelioma
No, 6 months
 
UK unknown
In conclusion, we encountered a case of adenomatoid mesothelioma of peritoneal origin. It is important to accumulate and share such experiences, which may lead to better characterization of this disease.

Acknowledgements

Not applicable.

Declarations

Not applicable.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Competing interests

The authors declare that they have no competing interests.
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Metadaten
Titel
Adenomatoid mesothelioma arising from the diaphragm: a case report and review of the literature
verfasst von
Kenta Kawabe
Hiroki Sato
Akiko Kitano
Ryuichi Yoshida
Kazuya Yasui
Yuzo Umeda
Kazuhiro Yoshida
Tomokazu Fuji
Kenjiro Kumano
Kosei Takagi
Masaaki Kagoura
Takahito Yagi
Toshiyoshi Fujiwara
Publikationsdatum
01.12.2022
Verlag
BioMed Central
Erschienen in
Journal of Medical Case Reports / Ausgabe 1/2022
Elektronische ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-022-03420-9

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