Int J Hematol (2015) 101:1–2 DOI 10.1007/s12185-014-1692-x
IMAGES IN HEMATOLOGY
Rhabdomyosarcoma infiltrating bone marrow Kriti Chauhan · Monica Jain · Pragya Shukla · R. K. Grover
Received: 11 August 2014 / Revised: 13 October 2014 / Accepted: 16 October 2014 / Published online: 28 October 2014 © The Japanese Society of Hematology 2014
Abstract We present a case of 26-year-old male, previously diagnosed as rhabdomyosarcoma (RMS) of perineal region. The peripheral smear showed a leukoerythroblastic picture with an occasional atypical cell. The bone marrow aspirate and biopsy showed monotonous sheets of malignant cells. On immunohistochemistry the tumor cells were strongly positive for desmin and negative for CD34 and CD117. This case illustrates the morphology and IHC findings in a case of RMS. Immunostains like CD34 and CD117 should be included to rule out a possibility of acute leukemia.
A 26-year-old male patient was presented with complaints of headache, vomiting and right lower limb weakness. On examination there was decrease in power of right lower limb (2/5) and left-sided upper motor neuron facial nerve palsy. The patient was previously diagnosed as a case of rhabdomyosarcoma (RMS) of perineal region and had received radiotherapy to pelvis up to a total dose of 45 Gy in 25 fractions followed by seven cycles of vincristine-, cyclophosphamide- and actinomycin D-based chemotherapy and three cycles of ifosphamide-, carboplatin- and etoposide-based chemotherapy. The patient underwent MRI brain which showed an ill-defined soft tissue density at the base of skull involving occipital bone extending along right jugular foramen. K. Chauhan · M. Jain (*) Department of Oncopathology, Delhi State Cancer Institute, Dilshad Garden, Delhi 110095, India e-mail: [email protected] P. Shukla · R. K. Grover Department of Clinical Oncology, Delhi State Cancer Institute, Dilshad Garden, Delhi 110095, India
His biochemical investigations revealed elevated alkaline phosphatase (ALP = 143 U/L), elevated lactate dehydrogenase (LDH = 2,794 U/L) and high uric acid levels (7.8 mg/dL). The complete blood count showed pancytopenia with hemoglobin = 7.4 mg/dL, total leukocyte count = 3.46 × 103/μL and platelet count = 10 × 103/μL. Peripheral smear examination showed a leukoerythroblastic picture with an occasional atypical cell. Based on his peripheral blood smear findings he underwent bone marrow aspiration and biopsy. The bone marrow aspirate smears revealed a cellular marrow with infiltration by blast-like cells with large round nuclei, reticulated chromatin with multiple nucleoli, moderate amount of basophilic cytoplasm and cytoplasmic vacuolation. Bone marrow biopsy showed near total replacement of marrow spaces by compact monotonous sheets of malignant cells in cords and tubules. On immunohistochemistry, the tumor cells were strongly positive for desmin and negative for CD34 and CD117, ruling out a hematological malignancy (Fig. 1a–c). The patient was planned for whole brain radiotherapy followed by chemotherapy, but the patient’s general condition persistently deteriorated and he expired prior to initiation of any palliative treatment. Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood and adolescence [1, 2]. The primary tumors are frequently found in head, neck or genitourinary tract. The tumor exhibits various histological variants, with embryonal and alveolar variants being the most common [3]. Approximately 16 %of patient with RMS has metastases at presentation and the bone marrow is involved in about 30 % of the cases [4]. RMS metastasizing to bone marrow closely mimics acute leukemia particularly of the lymphoblastic type [5]. The differentiation lies on a multidisciplinary approach using clinical findings, peripheral
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Fig. 1 a Bone marrow aspirate (Giemsa stain) ×100; b bone marrow (H&E stain) ×40; c IHC on bone marrow with Desmin (+)
smear, bone marrow examination, flow cytometry and immunohistochemistry, and is essential to predict the prognosis, survival and treatment of patients. Conflict of interest The authors declare that they have no conflict of interest.
References 1. Gurney JG, Young JL, Roffers SD, et al. Soft tissue sarcomas. In: Ries LAG, Smith MA, Gurney JG, et al., editors. Cancer incidence and survival among children and adolescents. Bethesda: NIH Publications; 1999. p. 1975–95.
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2. Hanke CA, Roessler J, Stegmaier S, et al. Alveolar rhabdomyosarcoma mimicking lymphoma with bone marrow involvement. Eur J Pediatr. 2007;166(5):505–6. 3. Boman F, Champigneulle J, Schmitt C, et al. Clear cell rhabdomyosarcoma. Pediatr Pathol Lab Med. 1996;16:951–9. 4. Jani Prashant, Charles CY. Massive bone marrow involvement by clear cell variant of rhabdomyosarcoma. Indian J Pediatr. 2009;76(2):224–8. 5. Sandberg AA, Stone JF, Czarnecki L, et al. Hematologic masquerade of rhabdomyosarcoma. Am J Hematol. 2001;68:51–7.
IMAGES IN HEMATOLOGY
Rhabdomyosarcoma infiltrating bone marrow Kriti Chauhan · Monica Jain · Pragya Shukla · R. K. Grover
Received: 11 August 2014 / Revised: 13 October 2014 / Accepted: 16 October 2014 / Published online: 28 October 2014 © The Japanese Society of Hematology 2014
Abstract We present a case of 26-year-old male, previously diagnosed as rhabdomyosarcoma (RMS) of perineal region. The peripheral smear showed a leukoerythroblastic picture with an occasional atypical cell. The bone marrow aspirate and biopsy showed monotonous sheets of malignant cells. On immunohistochemistry the tumor cells were strongly positive for desmin and negative for CD34 and CD117. This case illustrates the morphology and IHC findings in a case of RMS. Immunostains like CD34 and CD117 should be included to rule out a possibility of acute leukemia.
A 26-year-old male patient was presented with complaints of headache, vomiting and right lower limb weakness. On examination there was decrease in power of right lower limb (2/5) and left-sided upper motor neuron facial nerve palsy. The patient was previously diagnosed as a case of rhabdomyosarcoma (RMS) of perineal region and had received radiotherapy to pelvis up to a total dose of 45 Gy in 25 fractions followed by seven cycles of vincristine-, cyclophosphamide- and actinomycin D-based chemotherapy and three cycles of ifosphamide-, carboplatin- and etoposide-based chemotherapy. The patient underwent MRI brain which showed an ill-defined soft tissue density at the base of skull involving occipital bone extending along right jugular foramen. K. Chauhan · M. Jain (*) Department of Oncopathology, Delhi State Cancer Institute, Dilshad Garden, Delhi 110095, India e-mail: [email protected] P. Shukla · R. K. Grover Department of Clinical Oncology, Delhi State Cancer Institute, Dilshad Garden, Delhi 110095, India
His biochemical investigations revealed elevated alkaline phosphatase (ALP = 143 U/L), elevated lactate dehydrogenase (LDH = 2,794 U/L) and high uric acid levels (7.8 mg/dL). The complete blood count showed pancytopenia with hemoglobin = 7.4 mg/dL, total leukocyte count = 3.46 × 103/μL and platelet count = 10 × 103/μL. Peripheral smear examination showed a leukoerythroblastic picture with an occasional atypical cell. Based on his peripheral blood smear findings he underwent bone marrow aspiration and biopsy. The bone marrow aspirate smears revealed a cellular marrow with infiltration by blast-like cells with large round nuclei, reticulated chromatin with multiple nucleoli, moderate amount of basophilic cytoplasm and cytoplasmic vacuolation. Bone marrow biopsy showed near total replacement of marrow spaces by compact monotonous sheets of malignant cells in cords and tubules. On immunohistochemistry, the tumor cells were strongly positive for desmin and negative for CD34 and CD117, ruling out a hematological malignancy (Fig. 1a–c). The patient was planned for whole brain radiotherapy followed by chemotherapy, but the patient’s general condition persistently deteriorated and he expired prior to initiation of any palliative treatment. Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood and adolescence [1, 2]. The primary tumors are frequently found in head, neck or genitourinary tract. The tumor exhibits various histological variants, with embryonal and alveolar variants being the most common [3]. Approximately 16 %of patient with RMS has metastases at presentation and the bone marrow is involved in about 30 % of the cases [4]. RMS metastasizing to bone marrow closely mimics acute leukemia particularly of the lymphoblastic type [5]. The differentiation lies on a multidisciplinary approach using clinical findings, peripheral
13
2
K. Chauhan et al.
Fig. 1 a Bone marrow aspirate (Giemsa stain) ×100; b bone marrow (H&E stain) ×40; c IHC on bone marrow with Desmin (+)
smear, bone marrow examination, flow cytometry and immunohistochemistry, and is essential to predict the prognosis, survival and treatment of patients. Conflict of interest The authors declare that they have no conflict of interest.
References 1. Gurney JG, Young JL, Roffers SD, et al. Soft tissue sarcomas. In: Ries LAG, Smith MA, Gurney JG, et al., editors. Cancer incidence and survival among children and adolescents. Bethesda: NIH Publications; 1999. p. 1975–95.
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2. Hanke CA, Roessler J, Stegmaier S, et al. Alveolar rhabdomyosarcoma mimicking lymphoma with bone marrow involvement. Eur J Pediatr. 2007;166(5):505–6. 3. Boman F, Champigneulle J, Schmitt C, et al. Clear cell rhabdomyosarcoma. Pediatr Pathol Lab Med. 1996;16:951–9. 4. Jani Prashant, Charles CY. Massive bone marrow involvement by clear cell variant of rhabdomyosarcoma. Indian J Pediatr. 2009;76(2):224–8. 5. Sandberg AA, Stone JF, Czarnecki L, et al. Hematologic masquerade of rhabdomyosarcoma. Am J Hematol. 2001;68:51–7.
