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 Gynaecology, 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 child’s 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.

References

1.        Donegan Wl: Cancer and pregnancy. CA Cancer J Clin 1983;33:194-214.
2.        Stout AP. Rhabdomyosarcoma of the Skeletal Muscles. Ann Surg. 1946;123:447–472.
3.        Gurney JG, Young JL, Roffers SD, Smith MA, Bunin GR. SEER Pediatric Monograph. National Cancer Institute; 2005. Soft Tissue Sarcomas.
4.        Ries LAG, Smith MA, Gurney JG, Linet M, Tamra T, Young JL, Bunin GR, editors. Cancer incidence and survival among children and adolescents: United States SEER Program 1975–1995. Bethesda: National Cancer Institute;1999.
5.        Gurney JG, Severson RK, Davis S, et al.: Incidence of cancer in children in the United States. Sex-, race-, and 1-year age-specific rates by histologic type. Cancer 1995;75(8): 2186-95.
6.        Ries LA, Kosary CL, Hankey BF, et al., eds.: SEER Cancer Statistics Review, 1973-1996. Bethesda, Md: National Cancer Institute, 1999. 
7.        Grufferman S, Wang HH, DeLong ER, Kimm SY, Delzell ES, Falletta JM. Environmental factors in the etiology of rhabdomyosarcoma in childhood. J Natl Cancer Inst 1982;68:107–113.
8.        Grufferman S, Gula MJ, Olshan AF, Falletta JM, Pendergrass TW, Buckley J, Maurer HM. In utero x-ray exposure and risk of childhood rhabdomyosarcoma. Paediatr Perinat Epidemiol 1991;5:A6.
9.        Hartley AL, Birch JM, McKinney PA, Teare MD, Blair V, Carrette J, Mann JR, Draper GJ, Stiller CA, Johnston HE, et al. The Inter-Regional Epidemiological Study of Childhood Cancer (IRESCC): case control study of children with bone and soft tissue sarcomas. Br J Cancer 1988;58:838–842.
10.    10. Ghali MH, Yoo KY, Flannery JT, Dubrow R. Association between childhood rhabdomyosarcoma and maternal history of stillbirths. Int J Cancer 1992;50:365–368.

Citation

Zarariya A, Katke RD, Lewis P, Agrawal GD. Advanced Rhabdomyosarcoma during pregnancy. JPGO 2014 Volume 1 Number 8 Available from: http://www.jpgo.org/2014/08/advanced-rhabdomyosarcoma-during.html