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Case Reports
. 2024 Jul 18;12(7):164.
doi: 10.3390/diseases12070164.

Nerve Sheath Myxoma in Pregnancy: A Case Report

Elena De Chiara  1 , Valerio Gaetano Vellone  2 , Jacopo Ferro  2 , Chiara Trambaiolo Antonelli  2 , Liliana Piro  3 , Stefano Avanzini  3 , Valentina Prono  4 , Andrea Beccaria  5 , Monica Muraca  5 , Ramona Tallone  5
Affiliations
Case Reports

Nerve Sheath Myxoma in Pregnancy: A Case Report

Elena De Chiara et al. Diseases. .

Abstract

Nerve sheath myxoma (NSM) is a rare benign peripheral nerve sheath tumor that affects young adults. NSMs are asymptomatic, slow-growing swellings located in the upper extremities, more rarely in the lower extremities. Given the high risk of recurrence, it is recommended to perform a complete exeresis. To our knowledge, the evolution and management of NMS during pregnancy have not been described yet. We report the first case of recurrent pretibial NSM in a pregnant girl and its follow-up and outcome during and after pregnancy. NSM is difficult to diagnose clinically or using imaging. The final diagnosis remains histopathological. It is known how various types of benign and malignant skin tumors can develop or change during pregnancy. With our case, however, we documented that pregnancy does not affect the growth and evolution of NSM. Given the benign nature of the lesions and their tendency to grow slowly, during pregnancy, follow-up of NSMs can be conducted through ultrasonography and surgical treatment postponed after delivery. Our case highlights the importance of careful monitoring and individualized decision making, especially in rare scenarios such as NSM, where data on the progression of benign lesions are limited. Our case highlights the importance of a careful monitoring and a tailored treatment in rare scenarios such as NSM, where data on the progression of benign lesions are limited. Considering the benign nature of the lesions and their tendency to grow slowly, follow-up of NSMs during pregnancy can be conducted through ultrasonography, and surgical treatment can be postponed after delivery.

Keywords: immunohistochemistry; leg soft tissue tumor; nerve sheath myxoma; neurothekeoma; peripheral nerve sheath tumor; pregnancy.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Macroscopic (a) and radiological (b,c) appearance of the lesion, hard nodulation of approximately 2 × 1.5 cm, underlying smaller “cluster” lesions, MRI: nodulations uniformly hyperintense in STIR, ultrasound: hypoechogenic lesions, with polylobate margins, partially confluent, without vascularization upon color Doppler analysis.
Figure 1
Figure 1
Macroscopic (a) and radiological (b,c) appearance of the lesion, hard nodulation of approximately 2 × 1.5 cm, underlying smaller “cluster” lesions, MRI: nodulations uniformly hyperintense in STIR, ultrasound: hypoechogenic lesions, with polylobate margins, partially confluent, without vascularization upon color Doppler analysis.
Figure 2
Figure 2
Histologic examination of placenta (a,b) and tumor (c–e): (a) long-standing hypoxic distress with recent and organized intervillous and retroplacental hemorrhages and recent and organized intervillous and retroplacental hemorrhages. (b) Paracellular ischemic lesions with coagulative necrosis. (c) 6×. Dermo-ipodermal lesion with polynodular growth and expansile borders. (d) 40×. Thin fibrous capsule surrounding each nodule. (e) 100×. Spindle and stellate elements in myxoid and edematous stroma.
Figure 2
Figure 2
Histologic examination of placenta (a,b) and tumor (c–e): (a) long-standing hypoxic distress with recent and organized intervillous and retroplacental hemorrhages and recent and organized intervillous and retroplacental hemorrhages. (b) Paracellular ischemic lesions with coagulative necrosis. (c) 6×. Dermo-ipodermal lesion with polynodular growth and expansile borders. (d) 40×. Thin fibrous capsule surrounding each nodule. (e) 100×. Spindle and stellate elements in myxoid and edematous stroma.
See this image and copyright information in PMC

References

    1. Harkin J.C., Reed R.J. Tumors of the Peripheral Nervous System. Atlas of Tumo Pathology. Armed Forces Institute of Pathology; Washington, DC, USA: 1969. pp. 60–64. (Second Series, Fascicle 3).
    1. von Mehren M., Randall R.L., Benjamin R.S., Boles S., Bui M.M., Ganjoo K.N., George S., Gonzalez R.J., Heslin M.J., Kane J.M., et al. Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology. J. Natl. Compr. Canc Netw. 2018;16:536–563. doi: 10.6004/jnccn.2018.0025. - DOI - PubMed
    1. The WHO Classification of Tumours Editorial Board . WHO Classification of Tumours. 5th ed. IARC Press; Lyon, France: 2020. Soft tissue and bone tumours.
    1. Fetsch J.F., Laskin W.B., Miettinen M. Nerve sheath myxoma: A clinicopathologic and immunohistochemical analysis of 57 morphologically distinctive, S-100 protein- and GFAP-positive, myxoid peripheral nerve sheath tumors with a predilection for the extremities and a high local recurrence rate. Am. J. Surg. Pathol. 2005;29:1615–1624. doi: 10.1097/01.pas.0000173025.87476.a4. - DOI - PubMed
    1. Safadi R.A., Hellstein J.W., Diab M.M., Hammad H.M. Nerve Sheath Myxoma (Neurothekeoma) of the Gingiva, A Case Report and Review of the Literature. Head Neck Pathol. 2010;4:242–245. doi: 10.1007/s12105-010-0183-5. - DOI - PMC - PubMed

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