Malignant Schwannoma in Pregnancy:

A Case Report and Literature Review

Ann Kellogg, M.D.,1 and William J. Watson, M.D.2

ABSTRACT
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Malignant peripheral nerve sheath tumors in pregnancy are an uncommon

finding. This diagnosis in a pregnant patient with neurofibromatosis type 1 poses additional treatment dilemmas. A 28-year-old primigravida with known neurofibromatosis
type 1 complained of increasing shortness of breath and cough. Evaluation revealed a large
chest mass, biopsy proven to be a malignant schwannoma. This malignant peripheral nerve
sheath tumor, discovered at 26 weeks gestation, grew so rapidly that delivery was necessary
at 30 weeks gestation. Management of pregnant patients with rare, rapidly growing tumors
may require early delivery in cases where maternal health is in jeopardy.
KEYWORDS: Pregnancy, cancer, neurofibromatosis, nerve sheath tumor

eurofibromatosis is a rare disease. Individuals

with this diagnosis, however, have an elevated lifetime
risk of developing malignant peripheral nerve sheath
tumors. These tumors have the potential for rapid
primary growth and recurrence.
This case describes the rapid onset of symptoms
associated with a malignant peripheral nerve sheath
tumor in pregnancy. Treatment of the tumor necessitated early delivery of the fetus at 30 weeks gestation.

CASE REPORT
A 28-year-old Middle Eastern female, G1, P0, complained of cough and dyspnea that initially developed
during the pregnancy and was gradually increasing
over the past 2 months. Due to her symptoms, an
initial chest computed tomography (CT) scan was
done at an outlying hospital at 26 weeks gestation,
showing an enhancing mass in the left-upper mediastinum, extending from the base of the neck to the left
atrium (Fig. 1).
1
Obstetrics and Gynecology and 2Maternal-Fetal Medicine, Mayo
Clinic College of Medicine, Rochester, Minnesota.
Address for correspondence and reprint requests: William J.
Watson, M.D., Maternal-Fetal Medicine, Mayo Clinic College of
Medicine, Charlton 3B, 200 First Street SW, Rochester, MN 55905
(e-mail: watson.william@mayo.edu).
Am J Perinatol 2010;27:201204. Copyright # 2010 by Thieme

The past medical history was significant for neurofibromatosis type I. She also had a history of seizure
disorder, treated with carbamazepine, with no seizures in
the past 4 years.
Betamethasone therapy was given to enhance fetal
pulmonary maturity, and transfer to our facility was
made at 29 weeks gestation. Upon arrival, her symptoms
included cough, dyspnea, and fatigue. She denied contractions, leaking fluid per vagina, or vaginal bleeding
and had no abdominal pain; she had gained no weight
during the pregnancy. Obstetric ultrasound showed a
normal singleton intrauterine pregnancy with an estimated fetal weight of 1545 g. Cardiothoracic surgery and
oncology services were consulted. A CT-guided biopsy
revealed that the chest mass was a peripheral nerve
sheath tumor (schwannoma) with high-grade malignancy.
At first, the plan was to delay treatment of the
mass until 32 weeks gestation. Expectant management
was the initial choice in this case because of extreme
prematurity of the fetus and the poor prognosis for the
Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001,
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Received: March 11, 2009. Accepted after revision: June 8, 2009.
Published online: August 17, 2009.
DOI: http://dx.doi.org/10.1055/s-0029-1236439.
ISSN 0735-1631.

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AMERICAN JOURNAL OF PERINATOLOGY/VOLUME 27, NUMBER 3

Figure 1 Original chest computed tomography scan of the

patient. Left-upper mediastinum mass, extending from the
base of the neck to the left atrium.

mother with either immediate delivery or with expectant

management. Repeat imaging obtained 5 days after
admission was prompted by increasing maternal symptoms; the CT scan showed the left-upper mediastinal
mass increased in size, with new invasion of the left
anterior chest wall into the pectoralis muscle. There was
also a moderate-sized pericardial effusion. The mass
compressed the mediastinal structures to the right and
showed compression of the left main pulmonary artery.
Due to the rapid increase in the size of the mass,
the decision was made to proceed with immediate
delivery of the infant, so the patient could undergo
aggressive treatment. At 30 weeks gestation, a low
transverse cesarean delivery was performed under general
anesthesia without difficulty. The mother tolerated the
surgical procedure well and remained hemodynamically
stable while in the operating room. She was transferred
to the surgical intensive care unit, intubated for further
monitoring, but had an uneventful 4-day postoperative
course.
A live-born male infant was delivered from the
vertex presentation, weighing 1470 g. Apgar scores were
8 and 9. The infant was intubated immediately after
surgery and given surfactant due to respiratory distress
syndrome. Initial complications included hypotension,
resolved with fluid boluses, and jaundice, treated with
phototherapy. Head ultrasound on the infant was unremarkable. On day 7, enteral feeds were started, which
were tolerated well. On day 8, the infant was transitioned to continuous positive airway pressure (CPAP),
and on day 14, nasal CPAP was discontinued. The
infant was discharged home on day 36 in stable condition, weighing 2395 g.

2010

On postoperative day 4, the patient was started on

mitomycin, doxorubicin, and cisplatin chemotherapy
with a 25% dose reduction, due to the plan for concomitant radiation therapy, which commenced the following day. She was also treated with allopurinol to
attempt to prevent tumor lysis syndrome and prophylactic doses of heparin. A percutaneous endoscopic
gastrostomy tube was placed to improve nutrition due
to continued weight loss, odynophagia, and decreased
appetite.
After receiving her neoadjuvant therapy, the patient underwent surgery to remove the mass, with
excision of the anterior mediastinal mass and en bloc
resection of thymus, pericardium, a portion of left lung,
and a portion of the left phrenic nerve. The pathology
from the surgery was consistent with a previous biopsy,
showing high-grade malignant peripheral nerve sheath
tumor, predominantly necrotic. The excised segment
showed 2-cm tumor-free margins.
Subsequent chemotherapy, consisting of ifosfamide and adriamycin, was administered every 3 weeks
for a total of five cycles. The patient remained on
anticoagulation with coumadin for 3 months after her
tumor resection. The patient returned home in stable
condition.
A follow-up CT scan 6 months after discharge
showed multiple areas of metastasis including the left
iliac wing, right side of the L1 vertebral body, a nodule
on the inferior portion of the right kidney, left perirectal
nodules, left internal iliac lymph nodes, and a pelvic
sidewall mass. The patient expired approximately
1 month later.

DISCUSSION
Neurofibromatosis type I is a rare autosomal-dominant
disease, with a prevalence of 1/3000. This disease
affects chromosome 17q11.2, the NF-1 gene, which is
thought to play a role as a tumor suppressor gene. The
criteria for diagnosing neurofibromatosis type I includes
two of the following six signs: cafe au lait spots, two or
more neurofibromas, one relative with the diagnosis,
optic glioma, freckling, Lisch nodules, and distinctive
bony lesions. The lifetime risk of developing a malignant
peripheral nerve sheath tumor in a patient with neurofibromatosis is 4.6%.1 In patients with known neurofibromas, malignant degeneration can occur in 1 to 2%.2
These patients are at a fourfold increase of developing a
malignancy in their lifetime.3 Many times, patients with
this disorder present with large tumors and the prognosis
is poor. Nebesio et al noted that 50% of existing neurofibromas enlarge and 60% of new neurofibromas appear
during pregnancy.4 Clinicians should therefore have a
low threshold for considering a tumor, either benign or
malignant, in patients with neurofibromatosis who
develop unexplained symptoms.

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Table 1 Malignant Schwannomas in Pregnancy2,79

Age and
Reference

G/P

Gestational Age
at Presentation

Gestational Age
at Delivery

Location of Tumor

Outcome

28

G1, P0

26 wk

30 wk

Upper mediastinum

Radiation, chemotherapy
postpartum followed by
surgery; recurrence
<6 mo

362

G2, P1

1st

20 wkinduced due

Upper mediastinum

Surgery at 17 wk, followed

329

G3, P2

trimester
24 wk

to need for radiation

39 wk

Paravaginal mass, involving

by radiation after delivery

Surgery 6 wk postpartum;

198

G3, P1

175/7 wk

22 wksinduced

Anterior abdominal wall,

left ischiorectal fossa

after PPROM

involving upper

recurrence 7 mo later
Immediate surgery with
removal of mass

abdomen, vena cava,

wall, ribs
387

G4, P3

22 wk

36 wk

Ventral surface of forearm

Surgical resection at 29 wk,

radiation and
chemotherapy after
delivery

The first case in the table is the case presented in this report. PPROM, preterm premature rupture of membranes.

A few reports have been written about complications of benign schwannomas during pregnancy.
Although these tumors do not have the malignant nature
of the tumor presented in this case report, they can cause
difficulty due to compression of surrounding structures.
In one case, a benign esophageal schwannoma was noted
at 36 weeks gestation. After delivery and subsequent
tumor removal, no adjuvant treatment was needed, due
to the nature of the tumor.5 In patients who present with
such symptoms late in pregnancy, the best decision may
be to proceed with immediate delivery and subsequently
decide on the best management approach for the tumor.
In patients with retroperitoneal schwannomas presenting early in pregnancy, the best management option is
close observation with ultrasound or other imaging
modalities, including magnetic resonance imaging.6
Malignant peripheral nerve sheath tumors are
compromised of peripheral Schwann cells and fibroblasts. They have the potential for rapid primary growth
and recurrence. In addition to the rapid growth and
malignant potential of these tumors, there are additional
factors to consider if they occur during pregnancy. We
could find four previous case reports of malignant
peripheral nerve sheath tumors in pregnancy.2,79
This search was conducted on PubMed, from 1966 to
February 2009, with the search terms peripheral nerve
sheath tumor, schwannoma, and pregnancy. These
cases are summarized in Table 1.
With abdominal or pelvic tumors, obstetric complications can include uterine displacement as well as
compression of major vessels, leading to inadequate
uterine blood flow.8 Unfortunately, in our case, the

tumor was rapidly enlarging, located in the anterior

mediastinum, and causing compressive symptoms requiring immediate delivery. We decided that cesarean
section was the best option of delivery because of the
patients medical condition, gestational age, and unfavorable cervix. General anesthesia care was used for the
surgery, and preparation was made for cardiopulmonary
support during surgery, if needed. Due to the gestational
age at tumor presentation, our fetal outcome was good;
however, recurrence was noted clinically in less than
6 months after aggressive therapy.
Malignant peripheral nerve sheath tumors have a
poor prognosis. Most patients eventually experience a
tumor recurrence and succumb to the disease within a
year.7 Because the definitive management of these tumors is surgical, followed by radiation and/or chemotherapy, complete local excision of the tumor is
extremely important. Depending on the location of the
tumor, surgical excision during pregnancy can be difficult to accomplish. As demonstrated in Table 1, if
patients present very early in pregnancy, the likelihood
of achieving a successful pregnancy outcome is low, due
to the malignant nature of the disease and necessity to
proceed quickly to definitive management of the tumor
for the mothers health.
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