Logo American Journal of Case Reports

Call: 1.631.629.4328
Mon-Fri 10 am - 2 pm EST

Contact Us

Logo American Journal of Case Reports Logo American Journal of Case Reports Logo American Journal of Case Reports

13 February 2021: Articles  Italy

Diffuse Micro-Nodules on Peritoneal Surfaces at Donor Organ Procurement: Highlights on the Diagnostic Challenge and Transplant Management

Unusual setting of medical care, Rare disease

Albino Eccher1ABEF*, Amedeo Carraro2ABG, Ilaria Girolami1ACDEF, Manuela Villanova1EF, Alex Borin2BE, Paola Violi3EF, Barbara Paro3B, Claudia Mescoli4ABD, Deborah Malvi5BD, Luca Novelli6ABCD, Antonietta D'Errico5ABC, Giuseppe Rossini7ABD, Marco Ungari8AB

DOI: 10.12659/AJCR.929348

Am J Case Rep 2021; 22:e929348

0 Comments

Abstract

BACKGROUND: Guidelines have been designed to stratify the risk of cancer transmission in donors with a history of or ongoing malignancy, although this evaluation is not always straightforward when unexpected and rare lesions are found.

CASE REPORT: Here, we present a case of a 41-year-old African female donor who died from a cerebral hemorrhage. Her medical history was unavailable. At procurement, multiple diffuse grayish small nodules were noticed along the peritoneal cavity, some of which were sent to the on-call pathologist for urgent frozen section evaluation. Histology showed a multinodular proliferation of uniform bland-appearing spindle cells, with no evidence of necrosis, nor nuclear atypia or mitoses. The overall picture was consistent with the diagnosis of disseminated peritoneal leiomyomatosis, with overlapping morphology with uterine leiomyoma. Given the rarity of the lesion and the potential for recurrence or malignant degeneration, only the liver and heart were allocated to recipients with life-threatening conditions. The decision was taken in a forcedly limited time and took into account the benefit of transplantation and the risk of disease transmission.

CONCLUSIONS: This case highlights challenges that transplant teams often have to deal with, as lesions that are difficult to diagnose during donor assessment are usually not covered in guidelines. The acceptance and usage of organs in such cases has to be decided in a team-based fashion, with the collaboration of all the transplant professionals involved to optimally assess the transmission risk, carefully balancing the benefits of transplantation for the recipients and the need to guarantee a reasonable degree of safety.

Keywords: Leiomyomatosis, Organs at Risk, Transplantation, Liver, Liver Transplantation, Tissue Donors, Tissue and Organ Procurement

Background

The number of patients waiting for a transplant is rising and the availability of donor organs does not meet the needs. For this reason, so-called marginal donors are considered, including those with a history of ongoing malignancy. However, transplantation of an organ from a donor always carries a potential risk of disease transmission [1,2].

Guidelines and recommendations have been published in several countries [3–6] to stratify the risk of cancer transmission of donors with neoplastic potential. Guidelines are mainly based on large registries and sparse case reporting of transmission events, so they cannot cover all the possible findings and situations [7,8]. Indeed, evaluation of deceased donors at procurement can be critical when transplant professionals have to manage cases where an exhaustive medical history is not available or when investigations procedures could not be performed. Even when a reliable diagnosis of unexpected lesions is achieved, its rarity leads often to an uncertain risk assessment of the donor [9]. Our case shows an unexpected finding diagnosed as disseminated peritoneal leiomyomatosis, during the forced limited time of organ procurement, in a donor with no available history. Moreover, the transplant team was obliged to make a critical decision, balancing risks and benefits of using the grafts from a donor with a rare diagnostic entity not covered by guidelines.

Case Report

The organ donor was a 41-year-old African woman who died from a cerebral hemorrhage. A medical history was unavailable and assessment of the donor before organ procurement was performed according to Italian National Guidelines [5]. Blood tests were normal, serology was negative, and all clinical investigations were unremarkable. Ultrasound examination of head and neck and breast did not show any abnormalities, while total body computerized tomography revealed an enlarged, fibromatous uterine corpus. The kidneys, heart, liver, and lungs were allocated to 5 recipients at different transplant centers.

During abdominal exploration at procurement, multiple diffuse grayish small nodules of sub-centimetric size were noticed along the peritoneal cavity, both on the parietal and on the visceral side (Figure 1), with extensive involvement of the pre-renal space. The uterus was enlarged due to gray-ish, fasciculated nodules, the largest measuring 3 cm in diameter. Exploration of the thorax and abdomen ruled out any other sign of malignancy or infection. Some of the peritoneal nodules and the uterus were sent to the on-call patholo-gist for frozen section evaluation. Histology showed a multi-nodular proliferation of uniform bland-appearing spindle cells, with no evidence of necrosis, nor nuclear atypia or mitoses (Figure 2A, 2B). Taken together, the clinicopathological findings were suggestive of a mesenchymal proliferation without signs of malignancy, consistent with the diagnosis of disseminated peritoneal leiomyomatosis.

Transplant centers were advised to allocate the organs only to recipients in life-threatening conditions and to implement strict oncological surveillance after transplantation. The heart and liver did not show any alterations and were recovered and transplanted, while the presence of multiple nodules along the course of the ureter and in the pre-renal space precluded use of the kidneys. Immunohistochemistry subsequently performed on the formalin-fixed paraffin-embedded samples confirmed, in the early days after, the diagnosis of disseminated peritoneal leiomyomatosis (Figure 2C, 2D). The morphological appearance was indeed overlapping with uterine leiomyoma and no signs of malignancy were found in definitive samples. The recipients underwent a stricter oncological follow-up with more frequent clinical evaluation and imaging if additional elements of suspicion arise. At the time of writing, the recipients are doing well, without signs of disease related to the donor’s condition. This study was approved by the Institutional Ethics Committee AOUI Verona (ref. No. 1745CESC).

Discussion

The risk of malignancy transmission with transplant appears to be low according to large series and registries, but it is not zero [10–13]. While guidelines provide a clear indication on managing the organs from a donor with a history of or ongoing malignancy for most cancer types, this is not the case for the rarer and less-described lesions, such as peritoneal leiomyomatosis, whose malignant potential is unclear. Our case is paradigmatic of how an unexpected finding challenged the transplant team in a limited time from the establishment of diagnosis to the definition of the level of risk transmission and therefore the allocation of organs.

First of all, the routine clinical and instrumental investigations performed on the donor did not reveal any relevant abnormality. The small diffuse nodules were discovered during the abdominal exploration, with their number and the spreading appearance on surfaces of peritoneum giving the suspicion of a malignant process. Differential diagnoses comprised a carcinomatous spread of an unknown primary, an infection, or a systemic disease. Histological examination at frozen section showed the features of mesenchymal benign proliferation with spindle cells arranged in whorls and sometimes patternless with no evident cytological atypia, without mitotic figures or evidence of necrosis and hemorrhage. The overall picture of the lesion, together with the presence of a fibromatous uterus, was consistent with a disseminated peritoneal leiomyomatosis, as reported in other cases in the literature [13–15]. Some cases of disseminated peritoneal leiomyomatosis are associated with uterine leiomyomata, as in our case.

Speculations have been made on the possibility of an origin of the lesion after surgical manipulation or interventions for myomectomy [14–17] or caused by a hormonal abnormal estrogenic stimulation causing a subperitoneal mesenchymal stem cell metaplasia [14]. A genetic origin is also hypothesized in association with other diseases, in a model of autosomic dominant disease with incomplete penetrance [18]. Moreover, disseminated peritoneal leiomyomatosis is known to have the potential to recur [19,20] and a sarcomatous degeneration has been reported [21,22]. Approximately 150 cases are described in the literature and this is the first case encountered in the setting of limited time of organ donor procurement. The absence of donor history and of comparable managed cases in the literature, together with the rarity and the uncertain malignant potential of this condition, were the main challenges in assessing a correct risk of transmission for the recipients.

Furthermore, in these cases, it is even more important that a reliable diagnosis is provided and that the balance of risks and benefits of usage of the organs is discussed with a multidisciplinary approach, preferably with the consultation of experts in surgical pathology, to better define the level of risk [23,24]. Suitable organs may be erroneously discarded if the nature of the lesion is not reliably assessed, but organs coming from a donor with such a rare lesion should be carefully considered and used with closer follow-up of recipients. The guidelines of the National Transplant Centre and other institutions usually do not cover the specific case, given the rarity and the uncertain malignant potential of these lesions; consequently, the consultation of available experts in the field of pathology and oncology is advisable and should always be part of the decision-making process. Moreover, these decisions have to be made within very strict time constraints of the procurement in order to ensure the correct usage of organs, avoiding their damage or erroneous discard. At the end of the process, it was decided to transplant the organs without changing the recipient allocation, but advising to implement a stricter oncological follow-up in order to provide the benefit of transplantation and at the same time to guarantee the highest possible level of safety. Due to the potential of recurrence and malignant degeneration of disseminated peritoneal leiomyomatosis, transplant centers allocate the organs only to recipients in life-threatening conditions and implement strict oncological surveillance after transplantation. This is stated in the guidelines, but such cases stress once again the importance of transplant team discussion with access to expertise of all professionals involved to keep the opportunity to transplant an organ and the assure the highest possible degree of safety.

Conclusions

The case report reinforces the need for a team-based decision-making, with the participation of all transplant professionals involved, from the procurement surgeons to the pathologists and to the recipients’ healthcare providers.

References:

1.. Desai R, Collett D, Watson CJ, Cancer transmission from organ donors-unavoidable but low risk: Transplant J, 2012; 94(12); 1200-7

2.. Eccher A, Girolami I, Marletta S, Donor-transmitted cancers in transplanted livers: Analysis of clinical outcomes: Liver Transpl, 2020 [Online ahead of print]

3.. Nalesnik MA, Woodle ES, DiMaio JM, Donor-transmitted malignancies in organ transplantation: Assessment of clinical risk: Am J Transplant, 2011; 11(6); 1140-47

4.. , Transplantation of organs from deceased donors with cancer or a history of cancer Published 2014. Accessed February 6, 2020https://www.odt.nhs.uk/transplantation

5.. , [General criteria for evaluation of donor suitability adopted in Italy.] Published 2017. Accessed February 6, 2020. [in Italian]www.trapiantipiemonte.it

6.. , Guide to the quality and safety of organs for transplantation Published 2018. Accessed February, 6 2020https://www.edqm.eu/en/reports-and-publications

7.. Eccher A, Cima L, Ciangherotti A, Rapid screening for malignancy in organ donors: 15-year experience with the Verona “Alert” protocol and review of the literature: Clin Transplant, 2017; 31(9); e13045

8.. Eccher A, Boschiero L, Fior F, Donor kidneys with miliary papillary renal cell neoplasia: The role of the pathologist in determining suitability for transplantation: Ann Transplant, 2014; 19; 362-66

9.. Eccher A, Brunelli M, Gobbo S, Subepithelial pelvic hematoma (Antopol-Goldman Lesion) simulating renal neoplasm: Report of a case and review of the literature: Int J Surg Pathol, 2009; 17(3); 264-67

10.. Eccher A, Lombardini L, Girolami I, How safe are organs from deceased donors with neoplasia? The results of the Italian Transplantation Network: J Nephrol, 2019; 32(2); 323-30

11.. Novelli L, Mescoli C, Malvi D, A sticky, palpable area of the perinephric adipose tissue at organ donor procurement: Highlights on the diagnostic challenge and transplant management: J Nephrol, 2020; 33(6); 1377-79

12.. Eccher A, Girolami I, Motter JD, Donor-transmitted cancer in kidney transplant recipients: A systematic review: J Nephrol, 2020; 33(6); 1321-32

13.. Gaichies L, Fabre-Monplaisir L, Fauvet R, Leiomyomatosis peritonealisis disseminata: Two unusual cases with literature review: J Gynecol Obstet Hum Reprod, 2018; 47(2); 89-94

14.. Al-Talib A, Tulandi T, Pathophysiology and possible iatrogenic cause of leiomyomatosis peritonealis disseminata: Gynecol Obstet Invest, 2010; 69(4); 239-44

15.. Della Corte L, Fabozzi A, Morra I, Disseminated peritoneal leiomyomatosis 10 years after a laparoscopic myomectomy: A brief communication: Eur J Obstet Gynecol Reprod Biol, 2018; 224; 58-59

16.. Gerashchenko AV, Filonenko TG, Golubinskaya EP, Morcellation-induced leiomyomatosis peritonealis disseminata: A rare case report: Iran J Med Sci, 2019; 44(1); 60-64

17.. Xiao J, Zhang R, Teng Y, Disseminated peritoneal leiomyomatosis following laparoscopic myomectomy: A case report: J Int Med Res, 2019; 47(10); 5301-6

18.. Halama N, Grauling-Halama SA, Daboul I, Familial clustering of leiomyomatosis peritonealis disseminata: An unknown genetic syndrome?: BMC Gastroenterol, 2005; 5(1); 33

19.. Ferrario L, Zerbi P, Angiolini MR, Leiomyomatosis peritonealis disseminata: A case report of recurrent presentation and literature review: Int J Surg Case Rep, 2018; 49; 25-29

20.. Jain M, Puri V, Beniwal A, Recurrent diffuse peritoneal leiomyomatosis following leiomyomata uterus: J Obstet Gynecol India, 2014; 64(S1); 105-6

21.. Chiu H-C, Wu M-Y, Li C-H, Epithelial-mesenchymal transition with malignant transformation leading multiple metastasis from disseminated peritoneal leiomyomatosis: J Clin Med, 2018; 7(8); 207

22.. Syed M, Parida B, Mankeshwar T, Patil A, Imaging findings in a rare case of leiomyomatosis peritonealis disseminata with malignant transformation: Pol J Radiol, 2017; 82; 426-30

23.. Eccher A, Girolami I, Brunelli M, Digital pathology for second opinion consultation an donor assessment during organ procurement. Review of the literature and guidance for deployment in transplant practice: Transplant Rev (Orlando), 2020; 34(4); 100562

24.. Girolami I, Gambaro G, Ghimenton C, Pre-implantation kidney biopsy. Value of the expertise in determining histological score and comparison with the whole organ on a series of discarded kidneys: J Nephrol, 2020; 33(1); 167-76

In Press

12 Feb 2024 : Case report  USA

Erdheim-Chester Disease Occult on Radiographs and CT but Visible on MRI and PET

Am J Case Rep In Press; DOI: 10.12659/AJCR.941169  

0:00

12 Feb 2024 : Case report  USA

Surgical Treatment of Spontaneous Superficial Temporal Artery Arteriovenous Malformation: A Case Report

Am J Case Rep In Press; DOI: 10.12659/AJCR.942839  

13 Feb 2024 : Case report  Bahrain

Warfarin Woes: A Rare Case of Hemoperitoneum with Intramural Small Bowel Hematoma

Am J Case Rep In Press; DOI: 10.12659/AJCR.943519  

13 Feb 2024 : Case report  Portugal

Hemorrhagic Presentation in Primary Central Nervous System Lymphoma: A Case Study

Am J Case Rep In Press; DOI: 10.12659/AJCR.942951  

Most Viewed Current Articles

07 Mar 2024 : Case report  USA

Neurocysticercosis Presenting as Migraine in the United States

DOI :10.12659/AJCR.943133

Am J Case Rep 2024; 25:e943133

0:00

10 Jan 2022 : Case report  Germany

A Report on the First 7 Sequential Patients Treated Within the C-Reactive Protein Apheresis in COVID (CACOV...

DOI :10.12659/AJCR.935263

Am J Case Rep 2022; 23:e935263

19 Jul 2022 : Case report  Saudi Arabia

Atlantoaxial Subluxation Secondary to SARS-CoV-2 Infection: A Rare Orthopedic Complication from COVID-19

DOI :10.12659/AJCR.936128

Am J Case Rep 2022; 23:e936128

23 Feb 2022 : Case report  USA

Penile Necrosis Associated with Local Intravenous Injection of Cocaine

DOI :10.12659/AJCR.935250

Am J Case Rep 2022; 23:e935250

Your Privacy

We use cookies to ensure the functionality of our website, to personalize content and advertising, to provide social media features, and to analyze our traffic. If you allow us to do so, we also inform our social media, advertising and analysis partners about your use of our website, You can decise for yourself which categories you you want to deny or allow. Please note that based on your settings not all functionalities of the site are available. View our privacy policy.

American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923