Abstract
This case report highlights the occurrence of B- cell lymphoblastic lymphoma (B-LBL) as a solitary cutaneous lesion without an existing systemic involvement and should be kept in the differentials while dealing with cases presenting with a similar clinical picture. We report the case of a 13-year-old girl who presented with a painful, progressively enlarging swelling in right zygomatico-temporal region, clinically simulated a deep fungal infection/granulomatous lesion and turned out to be a case of B-LBL without any systemic involvement on further work up. This case is being reported to emphasize that B-LBL should be considered as a differential for an otherwise benign appearing persistent lesion in the head and neck region.
Keywords: B-cell lymphoblastic lymphoma, benign, cutaneous, non-Hodgkin lymphoma
INTRODUCTION
Lymphoblastic lymphoma (LBL) is a rare form of non-Hodgkin lymphoma (NHL), arising from precursor T or B lymphocytes comprising 5% of all NHL.[1] Out of these, approximately <20% of the cases have cutaneous LBL at the time of presentation.[1,2] Most of the already reported cases with cutaneous lesions represent secondary cutaneous involvement by existing systemic precursor B-cell lymphoblastic lymphoma (B-LBL) or leukaemia rather than a primary cutaneous disease.[3] The morphological features of lymphoblastic lymphomas are similar to acute lymphoblastic leukaemia.
A 13-year-old girl presented with a benign-looking painful swelling on the zygomato-temporal region since nine months which clinically simulated a deep fungal infection/granulomatous lesion and turned out to be primary B-LBL.
This case is being reported to emphasize that B-LBL should be considered a differential for an otherwise benign-looking persistent lesion in the head and neck regions.
CASE HISTORY
We report a case of a 13-year-old girl who presented with a painful, progressively enlarging swelling in the right zygomato-temporal region for nine months. There was no associated history of fever/diplopia/headache/vomiting and/or weight loss.
On clinical examination, the swelling was tender and measured 5 × 5 cm; the overlying skin was erythematous and fixed to the swelling [Figure 1].
Figure 1.
Swelling over right zygomato-temporal region, measuring 5 × 5 cm with overlying fixed erythematous skin
The total leukocyte count was 6400 cells/μL, and there were no abnormal cells in the peripheral smear.
Fine needle aspiration cytology was performed, and it microscopically showed a few atypical lymphoid cells following which a trucut biopsy was sent for histopathological examination; however, it was inadequate for opinion.
Later, magnetic resonance imaging (MRI) scan of the brain was performed, which revealed a well-defined lobulated extracalvarial homogeneously enhancing altered signal intensity lesion in the right frontotemporal convexity appearing isointense on T1 and hyperintense on T2 weighted images. No evidence of intraparenchymal extension or bone erosion was noted. The radiological examination was suggestive of extracalvarial meningioma/lymphoma.
Excision of the swelling was planned under general anaesthesia; tumour showed adhesions to the underlying tissues; frozen section was sent intraoperatively which showed a tumour composed of monomorphic population of medium-sized lymphoid cells suggestive of NHL. A portion of the tumour was sent for histopathological examination, and the lesion was closed by placing a partial thickness skin graft from the right thigh over the raw area.
Grossly, a skin covered tissue mass weighing 38 g and measuring 6.5 × 5.5 × 2 cm was received in the department of pathology. Externally the skin showed an ulcer measuring 1.5 × 0.8 cm, and the cut section showed a tumour composed of homogeneous grey–white areas [Figure 2a and b]. Microscopically, the sections showed thinned out epidermis with subepidermal oedema, haemorrhage and a grenz zone in the papillary dermis [Figure 3a]. The dermis showed nodules and cords of tumour cells extending into the subcutaneous tissue [Figure 3b], composed of diffuse infiltration of monomorphic population of medium-sized lymphoid cells with scant cytoplasm, round to irregular nuclei, fine chromatin and inconspicuous nucleoli [Figure 4]. Immunohistochemistry was performed; tumour cells showed diffuse positivity for CD20, CD99, Tdt [Figure 5a–c], and negativity for CD3 and MPO. Ki-67 proliferation index was 75% [Figure 5d].
Figure 2.
(a) Grossly, a skin covered tissue mass measuring 6.5 × 5.5 × 2 cm with overlying skin showed an ulcer measuring 1.5 × 0.8 cm. (b) Cut section of the mass showed a tumour composed of homogeneous grey–white areas
Figure 3.
(a) Microscopically, thinned out epidermis with subepidermal oedema and a grenz zone in the papillary dermis seen (H&E ×100). (b) The dermis showed nodules and cords of tumour cells extending into the subcutaneous tissue (H&E ×100)
Figure 4.
High power view showed diffuse infiltration of monomorphic population of medium-sized lymphoid cells with scant cytoplasm, round to irregular nuclei, fine chromatin and inconspicuous nucleoli (H&E ×200)
Figure 5.
(a) The lymphoid cells express diffuse nuclear positivity for Tdt immunostain (Tdt ×100). (b) Diffuse membrane positivity for CD99 (CD99 ×100). (c) Diffuse membrane positivity for CD20 (CD20 ×200) and (d) Ki67 proliferation index of 75% (Ki67 ×200)
Based on the microscopy and immunohistochemistry, a final diagnosis of ‘Non-Hodgkin Lymphoma–B-cell lymphoblastic lymphoma’ was furnished.
The patient was advised chemotherapy; however, she was lost to follow up.
DISCUSSION
Skin is the second most common site of extranodal NHL following the gastrointestinal tract, with an estimated annual incidence of 1:100,000.[4,5] Lymphoblastic lymphomas constitute approximately 3.5%–7% of all malignant lymphomas of the skin.[5,6] Precursor lymphoblastic lymphoma is a neoplastic proliferation of either precursor T or B cells. Both the subtypes have varying clinical presentation with involvement of either blood or bone marrow, termed leukaemia or involvement of solid tissues, termed lymphoma. According to the World Health Organization's Classification of Tumours: pathology and genetics of skin tumours, B-LBL secondarily involves the skin. Precursor B-LBL is an uncommon form of lymphoblastic lymphoma that accounts for less than 10% of all lymphoblastic lymphoma cases.[3,7] T-LBL, the comparatively commoner variant, shows a male predominance and B-LBL though thought to have a male predominance, but according to many of the previously reported cases cutaneous B-LBL shows a slight female preponderance.[8,9,10] B-LBL has a predilection for the head and neck regions.[8]
Our case presented with involvement of skin over the head as the primary extranodal lesion, but did not involve any other sites. Till date, 38 cases of primary cutaneous B-LBL without extracutaneous involvement have been reported in the literature[8] and this is the 39th case. According to Song et al.,[8] the median age of all the previously reported cases was six years at the time of diagnosis (range 2 months to 14 years), with a male to female ratio of 1:1.4.
Based on clinical and radiological imaging findings, these lesions with soft-tissue scalp swelling with/without cranial vault involvement and underlying intracranial extension can simulate meningioma and should be kept in the differentials as the treatment and prognosis of both the entities vary.
On microscopic examination, B-LBL can present a diagnostic challenge by virtue of its resemblance to other lymphoid malignant neoplasms and small round blue cell tumours involving the skin as given in Table 1.[11] The abnormal lymphoid cells in B-LBL typically express TdT, CD43, CD99 and HLA-DR and may occasionally express B-cell markers such as CD79a, CD19, CD22 and CD20. Surface immunoglobulin is usually absent; however, cytoplasmic μ heavy chains may be present. The present case showed diffuse positivity for CD20, CD99, Tdt and negativity for CD3 and MPO. The main differentials and histopathological features are enlisted in Table 1.
Table 1.
Histopathological diagnosis of B-cell lymphoblastic lymphoma
| Disease | Microcopy | Immunophenotyping |
|---|---|---|
| B-cell lymphoblastic lymphoma | Uniform cells with finely dispersed chromatin and inconspicuous nucleoli | TdT+, CD34+/-, CD43+, CD10+/-, CD79a+, CD19+, CD20-/+, CD99+/-, CD45 +/- |
| Blastoid mantle cell lymphoma | Resemble lymphoblasts, dispersed chromatin and high mitotic count | TdT-, CD5+, CD10-, BCL2+, cyclin D1+, CD4+/- |
| Burkitt lymphoma | Uniform cells with clumped chromatin and several medium-sized nucleoli, ‘starry sky’ pattern | TdT-, CD34-, CD19+, CD20+, CD22+, CD79a+, CD10+, BCL6, BCL2-, surface immunoglobulin+/CD4+/CD56+ |
| Diffuse large B-cell lymphoma | Large cells with vesicular nuclei and prominent nucleoli | TdT-, CD34-, CD1+, CD20+, CD79a+, CD10-/+ |
| Leukaemia cutis (myeloid/myelomonocytic) | Uniform blastic cells | TdT-/+, CD34+, MPO+, myeloid markers+, CD68+and B-cell markers - |
| Ewing sarcoma or primitive neuroectodermal tumour | Small round blue cells | TdT-, CD34-, CD43-, CD79a-, CD99+ |
Treatment of B-LBL includes aggressive multidrug systemic chemotherapy protocols. After completion of therapy, regular follow up is essential to monitor acute effects of the treatment and also to rule out relapse.[8] Our patient was advised chemotherapy; however, she was lost to follow up.
CONCLUSION
This case is being reported in order to emphasize the consideration of B-LBL as a differential both clinically and morphologically in cases presenting with solitary benign-looking persistent cutaneous lesions over the head and scalp area in spite of its rare occurrence. It becomes imperative to give a timely diagnosis because this condition though aggressive, responds well to aggressive multiagent chemotherapy.[4]
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.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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