Advanced Rhabdomyosarcoma During Pregnancy

Author Information

Ashish Zarariya, Rajshree Dayanand Katke, Preeti Lewis, Grishma.D.Agrawal

(Associate Professor, Medical Superintendant & Associate Professor, Assistant
Professor, Third Year Resident. Department of Obstetrics and Gynecology,
Cama And Albless Hospitals, Grant Government Medical College, Mumbai,
India.)

Abstract

We report an interesting case of advanced rhabdomyosarcoma (RMS) in a teenage

pregnancy leading to mortality. A 19 year old married girl presented with 8 months of
amenorrhea and a wart like perianal lesion. She was lost follow up for a month and
came in emergency in critical condition with septicaemia, hyperkalemia and acute renal
failure (ARF). The wart sized lesion had progressed in a month to gross perianal mass
which was extending inside pelvis up to the lower lumbar region. The patient
succumbed within 8 hours of admission. On post-mortem histopathological examination,
the lesion was diagnosed as a rhabdomyosarcoma.

Introduction

A case of rhabdomyosarcoma with near term pregnancy is a exceedingly rare event.

Cancer in pregnancy itself is relatively rare. Most frequently reported sites are breast,
head and neck, lymphomas and melenomas.[1] The origin of RMS is from tissue that
imitates normal striated muscle.[2] Common sites are head and neck, genitourinary tract,
thorax and abdomen. RMS is classified by international classification as embryonal,
botryoid, spindle cell, alveolar, and undifferentiated. Out of this the botryoid and spindle
cell types, considered to be the subtypes of embroyonal RMS, occur in 0-10 age group
and have superior prognosis.[3] Alveolar RMS has poor prognosis, and its incidence is
evenly distributed in 0-19 age group.[4]
RMS is classified by international classification as Embryonal and Alveolar.
Out of this Botryoid and Spindle cell, considered to be the subtypes of Embroyonal.[3]
Case report

A 19 year old Primigravida Unregistered, came to ANC OPD for

Registration with 7 months amenorrhea and perianal wart like growth.

Figure 1. Perianal lesion at the first visit.

Later after 1 month, the patient presented in a critical state in emergency, with
palpable 3-4 cm hard lump in the left breast, edema in both lower limb up to
thighs and indurated ulcerative growth with multiple grouped vesicles present
around anus. The growth was extending inside the pelvis with gross left inguinal
lymphadenopathy.
Figure 2. Perianal lesion at the second visit.

She had an intrauterine fetal death at 34-36 weeks. On per vaginal examination,
the cervix could not felt due to the growth. She was treated with supportive line of
management. Her investigations were suggestive of hyperkalemia, septicemia
and ARF. She died within 8 hours of admission. Her postmortem examination
showed a palpable lymph node in left inguinal region 3 cm in diameter, swelling
of the left breast with palpable lymph node 2 cm diameter, a 10x8 cm whitish
colored growth in the left lower lumbar region and left side of the pelvis. It
extended to the perianal region in the form of nodules. The uterus showed
features of acute myometritis with degenerated decidua.

Figure 3: Postmortem abdominal gross findings.

Histopathological examination of the tumor mass around the vertebral column

showed a high grade malignant tumor with small round tumor cells with rhabdoid
differentiation- a rhadomyosarcoma. A part of uterus showed infiltration and
abscess formation, acute myometritis and degenerated decidua.

Discussion

The incidence of RMS is very low. Due to its rarity and diagnostic diversity, very little is
known about the etiology of RMS. Several environmental factors have increased risk of
developing RMS, such as paternal cigarette smoking,[5] advanced maternal age and x-
ray exposure in utero,[6] maternal and childs antibiotic use,[7] stillbirths[8] and maternal
recreational drug use[9]. In addition genetic changes may also play an important role in
RMS development. Familial syndromes associated with inherited gene defects, like Li-
Fraumeni syndrome and neurofibromatosis, have been associated with RMS. [10] RMS
relative 5-year survival rates have not increased significantly over the past 30 years;
 RMS has one of the worst prognosis with high rates of mortalities. The diagnosis of a
rhabdomyosarcoma depends on recognition of differentiation of its cells toward skeletal
muscle cells. Immunohistochemical marker of rhabdomyosarcoma are MyoD1 and
Myogenin. In our case immunohistochemistry could not be done as it was a post-
mortem case and the facility was not available in our institute. Pertaining to our case a
diagnosis of malignancy must be kept in mind for a painful ulcerative growth in this age
group.Our patient presented as a teenage pregnancy and so the tumor was all the more
rapidly progressive in nature leading to catastrophic, life threatening events ultimately
resulting in untimely mortality of the patient.

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