|Year : 2017 | Volume
| Issue : 3 | Page : 152-154
Myofibroblastic sarcoma in a sickle cell anemia patient: A case report from Sokoto, North-Western, Nigeria
Ibrahim Abdulqadir1, Muhammad Alhaji Ndakotsu1, Abubakar Umar Musa1, Umar Mohammed2
1 Department of Haematology and Blood Transfusion, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria
2 Department of Histopathology, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria
|Date of Web Publication||17-Aug-2017|
Department of Haematology and Blood Transfusion, Usmanu Danfodiyo University Teaching Hospital, Sokoto
Source of Support: None, Conflict of Interest: None
Low-grade myofibroblastic sarcoma (LGMS) is a rare malignant tumor of soft tissue that commonly presents as a painless enlarging mass of the affected site. There are several reported cases of malignant cancers among sickle cell disease (SCD) patients, and to the best of our knowledge, there was no previously reported case of LGMS occurring in a known SCD patient in our environment. We report a 16-year- old patient with sickle cell anemia who presented with the left knee swelling that was initially managed as a benign infection, but a later histological examination revealed a diagnosis of LGMS.
Keywords: Case report, low-grade myofibroblastic sarcoma, Nigeria, sickle cell anemia
|How to cite this article:|
Abdulqadir I, Ndakotsu MA, Musa AU, Mohammed U. Myofibroblastic sarcoma in a sickle cell anemia patient: A case report from Sokoto, North-Western, Nigeria. J Clin Sci 2017;14:152-4
|How to cite this URL:|
Abdulqadir I, Ndakotsu MA, Musa AU, Mohammed U. Myofibroblastic sarcoma in a sickle cell anemia patient: A case report from Sokoto, North-Western, Nigeria. J Clin Sci [serial online] 2017 [cited 2020 Jan 26];14:152-4. Available from: http://www.jcsjournal.org/text.asp?2017/14/3/152/213085
| Introduction|| |
Low-grade myofibroblastic sarcoma (LGMS) is a rare malignant neoplasm of the soft tissue mainly seen in the head and neck region and extremities., It occurs at any age with slight male predominance and usually presents as a painless enlarging mass of the affected site., Several malignancies, both hematological and nonhematological have been previously reported among sickle cell disease (SCD) patients and some reports suggest increased risk of developing malignant neoplasm among individuals with SCD compared to individuals without SCD.,, The concern for increased risk for malignancy is further heightened with the widespread use of hydroxyurea in the management SCD despite its proven clinical benefit and safety., From review of the literature available to us, there is no previously reported case of LGMS in a patient with SCD either on hydroxyurea or not. We report LGMS of the left knee in a 16-year-old sickle cell anemia (SCA) patient that was initially managed as infection of the left knee joint.
| Case Report|| |
The patient was a 16-year-old male student referred to our clinic from a peripheral hospital with a 3-month history of swelling of the left knee. The swelling was soft and fluctuant with no associated pain, limitation of movement across the joint, or history of trauma of the affected joint. There was no swelling in any other part of the body. However, about 6 months before presentation, he had a similar swelling on the same site which was surgically excised at the referring center after unsuccessful antibiotics therapy.
He was diagnosed with SCA since childhood and was regularly attending follow-up clinic at University of Maiduguri Teaching Hospital until 2013, when relocated to Sokoto following Boko Haram insurgency. He was regularly taking routine medications (folic acid and proguanil), had several blood transfusions in the past and never had any painful crisis warranting hospital admission or placed on hydroxyurea. He was the only sickle cell patient among seven children in a polygamous family setting.
General physical and systemic examinations were normal except for the pallor and obvious sickle cell habitus. There was a circumferential, fluctuant, and nontender swelling with healed scar measuring about 20 cm × 16 cm in the widest diameter on the left knee area [Figure 1] and left inguinal lymphadenopathy.
Hemoglobin electrophoresis showed Hb SS, and initial hemogram revealed normocytic anemia with thrombocytosis [Table 1]. Routine blood chemistry tests were within normal limit and viral screens (HIV I and II, HBsAg, and HCV) were negative. Aspirate from the swelling yielded no growth after 24 h culture and was negative to Ziehl–Neelsen stain, but its cytology showed cells with round to oval nuclei and mild pleiomorphism on inflammatory background. Magnetic resonance imaging showed encapsulated soft tissue mass communicating with the knee joint and no involvement of the underlying bones [Figure 2]. Histology of the mass revealed diffuse infiltrating sheets of spindle cells with mildly pleiomorphic hyperchromatic nuclei and moderate eosinophilic cytoplasm disposed in fibrocollageneous stroma [Figure 3]a. Immunohistochemistry of tumor was strongly positive to Vimentin [Figure 3]b demonstrated by showing dark-brown staining of the nuclei and negative for desmin [Figure 3]c and E1/AE2 [Figure 3]d demonstrated by showing no stains on the cytoplasm.
|Figure2: Magnetic resonance imaging of the left knee in a 16-year-old sickle cell anemia patient|
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|Figure3: Histology sections of the lesion.(a) Sheets of spindle cells with mildly pleomorphic hyperchromatic nuclei and moderate eosinophilic cytoplasm disposed in fibrocollageneous stroma (HandE, ×100), (b) Vimentin strongly positive; demonstrated by showing dark-brown staining of the nuclei of the tumor cells(Immuno, ×100),(c) desmin, and(d) AE1/AE2: The tumor cells were negative; demonstrated by showing no stains on cytoplasm of the tumor cells(Immuno, ×100)|
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| Discussion|| |
Myofibroblastic sarcoma is a rare low-grade soft tissue sarcoma with a predilection to head and neck region in about one-third of all cases and sometimes to extremities as seen in the index case., On histology, the tumor is composed mostly of spindle-shaped cells with ovoid or tapered nuclear and scanty or moderate amount of eosinophilic cytoplasm and stains positive to at least one myoid marker on immunohistochemistry such as Vimentin in the index case. LGMS is treated by surgical excision of lesion, though high incidence of recurrence has been observed.,,
Several malignancies were reported in the literature among SCD patients, and these include Hodgkin lymphoma, Non-Hodgkin lymphoma, acute and chronic leukemias, multiple myeloma, malignant fibrous histiocytosis, osteosarcoma, Wilms tumor, cancers of colon, lung, breast, prostate, renal, thyroid, and testes among others.,,,, So far, there is no precise association between SCD and any form of cancer, but both increased and reduced risk of developing cancer were suggested among individual with SCD., However, some conditions in SCD such as persistent inflammation, recurrent infections, blood transfusions, and use of hydroxyurea may be important in the pathogenesis of cancer and all, but the usage of hydroxyurea are applicable to our patient. To the best of our knowledge, there is no previously reported case of myofibroblastic sarcoma in a known SCD patient; hence, the report of this index case will create awareness and highlight on the occurrence of LGMS in SCD patients.
The symptoms of soft tissue sarcoma such as myofibroblastic sarcoma depend on the sites involved. Involvement of the lower limbs can be easily mistaken for more common benign conditions such as osteomyelitis or septic arthritis, especially when close to joints as was encountered in the index case. This may also be more likely in SCD patients as they are prone to both osteomyelitis and septic arthritis. Although presence of pain and other features of inflammation favor infectious rather than malignant conditions, it is worthy to note that about one-third of cases of soft tissue sarcoma may present with pain. This may pose diagnostic challenge. Similarly, in SCD patient there may be coexisting bone pain crises involving the affected site at the time of presentation, and this could mask the soft tissue sarcoma which most often present as nonpainful swelling of the affected site.
| Conclusion|| |
We have presented a 16-year-old sickle cell patient with a very rare low-grade soft tissue sarcoma (LGMS) which was initially managed as a benign infective condition that is prevalent among sickle cell patients without clinical improvement. High index of suspicion and histological examination of any excised body lesion will go a long way in early detection of rare tumors such as LGMS; thus, paving way toward reducing the attendant morbidity and mortality.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]