GYNECOLOGIC ONCOLOGY 34, 237-239 (1989)
CASE REPORT
Neurofibrosarcoma Complicating Pregnancy
VICKI V. BAKER, M.D. ,’ KENNETH D. HATCH,
Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, University of Alabama at Birmingham,
Birmingham, Alabama 35294
Received May 16, 1988
Sarcomatous degeneration of a neurolibroma during pregnancy
is exceedingly rare. Prompt diagnosis followed by surgical reset-
tion is recommended. o 1989 AC&& RCSS,IX.
Neurofibromatosis is an autosomal dominant disease
with an estimated incidence of 1:2000 to 1:3OOO[1,2].
The disease may exhibit a broad spectrum of associated
manifestations, ranging from unsightly but otherwise
asymptomatic cutaneous lesions to malignant degener-
ation of the neurofibromas. This disease is of interest to
the obstetrician-gynecologist not only because of the
need for appropriate genetic counseling but because of
the potential complications which may occur during
pregnancy.
Reported is a case of sarcomatous degeneration of a
pelvic neurofibroma associated with pregnancy. This is
only the second case reported in the English language
literature.
CASE REPORT
P.M. is a 32-year-old G3P3 black female diagnosed
with neurofibromatosis at age 13. Her family history was
positive for this disease.
The first two term pregnancies were uncomplicated.
During her third pregnancy, she did well until 24 weeks
estimated gestational age at which time she developed
left buttock pain and a vaginal mass. Physical exami-
nation revealed a 4 x 5-cm paravaginal mass that ex-
tended into the left ishiorectal fossa. This mass, thought
to represent a benign enlarging neurofibroma, was fol-
’ To whom requests should be addressed at Division of Gynecologic
Oncology, Department of Obstetrics and Gynecology, OHB 550, UAB
Station, University of Alabama at Birmingham, Birmingham, AL
35294.
M.D., AND HUGH M. SHINGLETON, M.D.
lowed during the remainder of the pregnancy. At 39
weeks estimated gestational age, the mass was obstruct-
ing the pelvic outlet and a cesarean delivery was per-
formed. Needle biopsy of the massat the time of delivery
revealed a spindle cell tumor consistent with a sarcoma.
A CT scan of the abdomen and pelvis obtained post-
operatively revealed a 14 x 12-cm encapsulated mass
which displaced the vagina across the midline (Fig. 1).
Bone involvement and intra-abdominal metastaseswere
not noted.
Six weeks postpartum, an 8 x 10 x B-cm mass par-
tially encapsulated by a thin fibrous capsule was re-
moved. A 4 x S-cm hemorrhagic cavity was present.
Microscopic examination demonstrated a high grade neu-
rofibrosarcoma (Fig. 2). The neoplasm was estrogen and
progesterone receptor negative.
The patient did well for 7 months at which time she
again noticed left buttock pain. Physical examination re-
vealed a firm, fixed lobulated mass at the prior site of
excision which extended from the level of the anus to
the left lateral vaginal fomix. CT scan demonstrated a
4 x S-cm ill-defined mass in the left ischiorectal fossa
(Fig. 3). Complete surgical resection of the recurrent
tumor was not achieved. Microscopic evaluation again
demonstrated histologic features consistent with a
neurofibrosarcoma.
DISCUSSION
The neurofibromatosis-related complications which
may occur during pregnancy have been recently re-
viewed [3]. Benign enlargement of neurofibromas during
pregnancy is not uncommon [4,5]. Dystocia secondary
to pelvic neurofibromas has also been previously re-
ported [2,6,7].
The overall incidence of malignant degeneration of
237
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238 BAKER, HATCH, AND SHINGLETON

FIG. 1. A large, encapsulated, predominantly homogeneous mass occupied the left ischiorectal fossa. Adjacent fat and muscle planes are
not well-visualized.

neural supporting tissues has been estimated to range
from 2.4 to 29% but only one prior case of sarcomatous
degeneration of a neurofibroma occurring during preg-
nancy has been reported [8,9]. Suggested but unproven
factors which may contribute to the malignant degen-
eration of a neurofibroma include trauma, prior surgery,
and a genetic predisposition. There is little evidence that
the high estrogen levels associated with pregnancy ac-
celerate the growth of soft tissue sarcomas [lo]. It is
unlikely that the hormonal milieu of pregnancy altered
the biologic behavior of this neurofibrosarcoma in view
of the absence of tumor estrogen and progesterone
receptors.
The diagnosis of sarcomatous degeneration of a neu-
rofibroma during pregnancy can be difficult. Complaints
of pain and a rapidly enlarging mass may be associated
with benign enlargement as well as malignant degener-
ation. The observation of a low density region within a
higher density mass by CT scan has been correlated with
sarcomatous degeneration of a neurofibroma [ 113. Hem-

FIG. 2. The resected mass consisted of malignant spindle cells with frequent mitotic figures. (H & E, 200 x )
 CASE REPORT
FIG. 3. The left ischiorectal fossa contained a 4 x S-cm mass that abutted the gluteus maximus muscle with extension of a less well-
defined mass adjacent to the rectum.
orrhage into a neurofibroma, which may occur during
pregnancy, results in a similar radiographic appearance
and diminishes the specificity of this finding [4,12].
Because the treatment of neurofibrosarcoma is pri-
marily surgical, the best results are achieved with small-
volume disease which can be completely resected. Fol-
lowing incomplete resection, these lesions tend to recur
locally although metastastic disease has also been re-
ported. The role of adjunctive chemotherapy and radia-
tion therapy is not clear largely because of the limited
number of reported cases treated in a variety of ways.
It is important for physicians to recognize the in-
creased risk of cancer in patients who have neurofibro-
matosis so that delays in diagnosis, with attendant dis-
ease progression, do not occur.
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