|Year : 2019 | Volume
| Issue : 2 | Page : 177-179
Retroperitoneal spindle cell variant of embryonal rhabdomyosarcoma in a child
Aditya Pratap Singh1, Arun Kumar Gupta1, Arpita Jindal2, Kalpana Mangal2
1 Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India
2 Department of Pathology, SMS Medical College, Jaipur, Rajasthan, India
|Date of Submission||15-Apr-2018|
|Date of Acceptance||07-Jun-2018|
|Date of Web Publication||25-Mar-2019|
Aditya Pratap Singh
Near The Mali Hostel, Main Bali Road, Falna, Dist-Pali, Rajasthan
Source of Support: None, Conflict of Interest: None
Spindle cell rhabdomyosarcoma (RMS) is a rare variant of embryonal RMS that has a predilection for young males and most commonly involves the paratesticular region followed by the head and neck. Histopathology reveals elongated spindle cells with fusiform to cigar-shaped nuclei and indistinct eosinophilic cytoplasm arranged in fascicles or whorls. Immunohistochemical workup demonstrates sarcomeric differentiation with reactivity for desmin, myogenin, and MyoD1 markers. Compared with other subtypes, the spindle cell variant in children is associated with a favorable outcome; however, in the adult population, there does not appear to be any prognostic advantage. We are presenting here a case of an embryonal variant of the spindle cell RMS in a 30-month-old male child.
Keywords: Embryonal, retroperitoneal, rhabdomyosarcoma, spindle
|How to cite this article:|
Singh AP, Gupta AK, Jindal A, Mangal K. Retroperitoneal spindle cell variant of embryonal rhabdomyosarcoma in a child. Med J DY Patil Vidyapeeth 2019;12:177-9
|How to cite this URL:|
Singh AP, Gupta AK, Jindal A, Mangal K. Retroperitoneal spindle cell variant of embryonal rhabdomyosarcoma in a child. Med J DY Patil Vidyapeeth [serial online] 2019 [cited 2019 Jun 16];12:177-9. Available from: http://www.mjdrdypv.org/text.asp?2019/12/2/177/254778
| Introduction|| |
Rhabdomyosarcoma (RMS) is the most common soft-tissue tumor of childhood. It is present in 50% of the all soft-tissue sarcoma in children. SC-RMS showed a strong predilection for young patients (mean age approximately 7 years) in the paratesticular region followed closely by the head and neck. In adults, the tumor reveals a preference for the head-and-neck region with a minority of cases arising in other locations such as subcutaneous and deep soft tissues of the extremities.
| Case Report|| |
Parent of a 30-month-old male child presented to us with the complaint of retention of urine. There was history of the difficulty in passing urine for the past 4 months. On local examination, there was a palpable lump in the lower abdomen. It was hard in consistency and immobile. Lump was noticed by mother 10 days back. Lump was slowly increasing in size thereafter. On general examination, the patient was poorly built and malnourished with pallor present. The size of the palpable lump was 5 cm × 5 cm. Patient was catheterized with Foley's catheter. Ultrasonography (USG) of the abdomen and pelvis suggestive of a large (100 mm × 53 mm) mixed echogenic mass was seen in the right iliac fossa extending to midline, pushing bladder left laterally with vascularity. Mass was encasing the anterior iliac artery and causing compression over bladder, leads to mild hydroureteronephrosis. Few retroperitoneal nodes were enlarged. Computed tomography scan of the abdomen suggestive of large heterogeneous (62 mm × 47 mm × 93 mm) density lesion was noted in pelvis anterior to urinary bladder extending on the right lateral side of urinary bladder [Figure 1]a and [Figure 1]b. It was displacing rectum and urinary bladder on the left side. It was abutting bilateral pubic symphysis with irregular destruction of underlying bilateral superior pubic ramus. There was mild dilatation of bilateral pelvicalyceal system and upper ureter is noted. There was also bilateral basal lung field that shows multiple nodular lesions? metastasis. It suggested of soft-tissue sarcoma. USG-guided tru-cut biopsy was done which showed spindle cell sarcoma [Figure 1]c and [Figure 1]d. Immunohistochemistry performed which showed that tumor cells exhibit immunopositivity for myogenin/desmin, smooth muscle actin, and vimentin and are immunonegative for CD 34, S-100, and MSA which confirmed the diagnosis of embryonal rhabdomyosarcoma [Figure 1]e and [Figure 1]f. As mass was fixed to pelvic vasculatures, it was impossible to operate, and so the patient was planned for chemotherapy. Chemotherapy regime vincristine, actinomycin-D, and cyclophosphamide (VAC) was started. Eight cycles of the VAC regimen were given. Thereafter, the patient started to pass urine spontaneously and size of the lump was decreased [Figure 2]a. We did contrast-enhanced computed tomography abdomen, it showed 49 mm × 50 mm × 62 mm size [Figure 2]b and [Figure 2]c. We planned for the surgical excision due to decrease in size. The mass was excised completely [Figure 2]d. Histopathology and immunohistochemistry showed embryonal RMS. Postoperative chemotherapy was given. Patient is under follow-up for the past 6 months without recurrence and doing well with improvement in the built and nourishment.
|Figure 1: (a and b) Prechemotherapy contrast-enhanced computed tomography images, (c and d) Histopathology slides, (e and f) Immunohistochemistry slides|
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|Figure 2: (a) Postneoadjuvant chemotherapy photo, (b and c) Postchemotherapy contrast-enhanced computed tomography images. (d) peroperative photo|
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| Discussion|| |
RMS can occur at any site although it is commonly affect in the head-and-neck region, genitourinary tract, and retroperitoneum and rarely to the extremities. In our case, it was arising from the retroperitoneum.
RMS has three histologic subtypes: embryonal, alveolar, and pleomorphic. The embryonal subtype, which is the most common, has three variants, spindle cell, botryoid, and anaplastic and representing up to 60%–80% of tumors at above sites.
Embryonic subtypes of RMS rarely involve the regional lymph nodes. RMS most often present as an expanding mass, resulting in pain and symptoms related to the compression of nearby structures. In our case, it was compressing the bladder leads to urinary symptoms.
Even though embryonal RMS is common, the spindle cell variant is considered rare. Our case was also spindle cell variant.
Tumors located in the paratesticular region allow for earlier detection, whereas pelvic tumors tend to be larger, and hence, paratesticular RMS have a better prognosis. Spindle cell RMS can be either well or poorly circumscribed, and there are no specific macroscopic features to distinguish this tumor.
Histologically, the tumor is composed mainly of elongated spindle cells arranged in a fasciculated or storiform pattern, mimicking smooth muscle fibers. Mitotic figures are easily appreciated including atypical forms. Admixed with the spindle cells, is the second population of immature rhabdomyoblasts, usually comprising a small population of the tumor. The presence of these cells should be an intimation of the diagnosis.
The diagnoses of most pediatric solid tumors require extensive immunohistochemical markers because they often exhibit a nonspecific small round-cell tumor phenotype. On immunohistochemistry, embryonal RMS may express vimentin, desmin, actin, myoglobin, myosin, creatine kinase M, titin, dystrophin, and acetylcholine receptor antigen.
RMS is treated by a combination of surgery, chemotherapy, and radiation. If the tumor is surgically inoperable, then initially radiotherapy and chemotherapy given to shrink a tumor followed by wide surgical excision of the tumor performed. Chemotherapeutic agents commonly used are vincristine, cyclophosphamide, dactinomycin, adriamycin, ifosfamide, and VP-16.
| Conclusion|| |
The spindle cell variant of embryonal RMS is a rare variant of an embryonal subtype of RMS, occurring more commonly in children and adolescents. Its low malignant potential and better prognosis in children makes the correct diagnosis crucial using a combination of clinical features, histopathology, and immunohistochemistry.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
The authors would like to thank Dr. Maryem Ansari, Pathologist, Assistant professor, SMS Medical College, Jaipur, Rajasthan, India.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]