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01.12.2017 | Case report | Ausgabe 1/2017 Open Access

BMC Cancer 1/2017

A case report of primary unilateral adrenal NK/T cell lymphoma: good clinical outcome with trimodality treatment

Zeitschrift:
BMC Cancer > Ausgabe 1/2017
Autoren:
Liu Hu, Weimin Xu, Mingwei Wang, Pan Wang, Guang Han, Chi Lin
Abbreviations
ACTH
Adrenocorticotropic hormone
CHOP
Cyclophosphamide, doxorubicin, vincristine and prednisolone
CISH
Chromogenic in situ hybridization
CNS
Central nervous system
CT
Computed tomography
EBV
Epstein-Barr virus
MRI
Magnetic resonance imaging
NK/T cell
Natural killer/T cell
SMILE
dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide

Background

There have been seven reported cases of NK/T cell lymphoma involving adrenal masses worldwide [ 17]. Among them, only one was primary adrenal NK/T cell lymphoma [ 2]. There have been no reported cases of the primary adrenal NK/T-cell lymphoma in China. Anatomically, it usually originates in the nasal cavity/nasopharynx and invades the surrounding tissues. Sometimes, it can arise in other organs including the skin, spleen/liver, or the gastrointestinal tract [ 8]. Here, we report a primary unilateral adrenal NK/T cell lymphoma with subsequent management. Although the longest survival was reported to be 90 days for adrenal NK/T cell lymphoma, our patient has been disease-free for 26 months.

Case presentation

Case report

A 28-year-old male experienced abdominal pain and a mass on the left side of the abdomen. Color duplex ultrasonogram and subsequent computed tomography (CT) scan revealed a 10 × 9 cm adrenal mass invading the top of left renal and partial of retroperitoneal region (Fig.  1. a and b). He underwent surgical removal of the mass in an outside hospital. The pathology specimens were evaluated in our hospital which showed neoplastic infiltration of pleomorphic lymphoid cells with scanty cytoplasm, irregular nuclear contour, and prominent nucleoli. Immunohistochemistry analysis showed that the tumor cells were positive for CD2, Bcl-2, CD43, CD56, negative for CD20, CD4, CD5, CD7, CD8, CD138, PCK, HMB45, Des, Pax-5, Bcl-6, Vim, CD34, TdT, MPO and equivocal for CD3. Ki67 labelling index was 40% (Fig.  2). Chromogenic in situ hybridization (CISH) was positive for the EBER gene (Fig.  3). These are consistent with NK/T-cell lymphoma.
After the surgery, the patient was without any systemic symptoms. He did have a 10 kg weight loss secondary to the removal of the majority of the large mass and the poor postoperative nutrition support. The postoperative physical examination was unremarkable. A postoperative CT scan of the abdomen showed a residual tumor, measuring 2.7 × 5.3 cm at the left side of retroperitoneum with the absence of left adrenal gland and kidney (Fig.  1. c). The bone marrow biopsy was negative. Nasal magnetic resonance imaging (MRI) showed normal findings. The contrast CT scans of the neck, chest and pelvis had been done and showed normal tissue structure. Primary unilateral adrenal NK/T cell lymphoma was diagnosed. He received four cycles of chemotherapy with cyclophophamide, doxorubicin, vincristine and prednisolone (CHOP). A post-chemotherapy CT scan revealed no residual disease (Fig.  1. d). He then underwent a course of consolidated intensity-modulated radiotherapy to the surgical bed to a dose of 50Gy in 25 fractions. Post-radiotherapy, the patient received two more cycles of CHOP. The patient tolerated treatment well without significant side effects. At the 26 months follow up point, the patient remains clinically and symptomatically disease-free.

Discussion

Primary adrenal lymphoma or lymphoma involving mainly the adrenal gland without the regional lymph node involvement is a very rare extra-nodal lymphoma. Patients with adrenal lymphoma may present with systemic symptoms including fever, fatigueand weight loss [ 911]. Primary adrenal lymphoma can be discovered incidentally on abdominal imaging. Differential diagnoses include cortical adenoma, pheochromocytoma, metastatic-disease, and adrenocorticotropic hormone (ACTH) dependent Cushing’s syndrome [ 12, 13]. The definitive diagnosis of primary adrenal lymphoma is made by an image-guided percutaneous biopsy, surgical exploration, or a postmortem examination [ 1, 2, 14]. Histopathologically, the most common type of primary adrenal lymphoma is B-cell type, mainly diffuse large B-cell lymphoma. Although involvement of the adrenal glands can be seen in disseminated disease, primary NK/T cell lymphoma in the adrenal gland as an initial presentation is extremely rare. The NK/T cell lymphomas are aggressive lymphoproliferative disorders derived from either activated NK cells or cytotoxic T cells. The 2008 World Organization classification system categorized NK–cell malignancies into three types: extra-nodal NK/T-cell lymphoma (nasal and non-nasal), aggressive NK-cell leukemia and chronic lymphoproliferative disorder of NK cells [ 15]. NK/T-cell lymphomas demonstrate vascular destruction with prominent necrosis. Immunophenotypically, they express CD2 and CD56. NK/T-cell lymphomas are strongly associated with Epstein-Barr virus (EBV), suggesting a pathogenic role of this virus [ 16, 17]. In this case, the patient’s tumor was positive for CD2, CD56 and EBER.
NK/T cell lymphoma is an extra-nodal lymphoma with predilection for the nasal cavity, nasopharynx, skin, and the other extra-nodal sites. The primary adrenal NK/Tcell lymphoma is exceedingly uncommon. In the review of the literature, we found only seven reported cases of NK/T cell lymphoma presenting as adrenal masses. Among these cases, the adrenal mass was the only involved site in one case [ 9]. The rest involved multiple organs, including central nervous system (CNS), paranasal sinus, kidney, bone marrow, etc. [ 1, 37]. Tissue diagnosis was made from the adrenal mass in four cases through adrenalectomy, CT-guided biopsy and autopsy specimen [ 13, 16], from cervical lymph nodes or nasal mass in two cases. There was no pathologic diagnosis in one case (the diagnosis was made per imaging study). The ages of the patients ranged from 17 to 76. Among these cases, the highly aggressive nature of the lymphoma and its rapid clinical progression were reported and the long-term prognosis has not been observed (Table.  1).
Table 1
Summary of patients with adrenal NK/T cell lymphoma
Authors
Year
Age
Sex
Organs invasion
Bilateral or unilateral
Type of specimen
Treatment
Survival
EBV positive
Dunning et al. [ 1]
2009
65
male
Bone marrow
unilateral
CT-guided biopsy
High-dose intravenous dexamethasone
(Died) few days after the final pathologic diagnosis
Yes
Thompson et al. [ 2]
2007
35
male
None
bilateral
Adrenalectomy
Surgery and chemotherapy (CHOP)
(Died) 90 days after the final pathologic diagnosis
Yes
Mizoguchi et al. [ 3]
2005
17
male
No mention
bilateral
Autopsy specimen
Immunosuppression therapy or plasma exchange
(Died) 4 days after admission
Yes
Toba et al. [ 4]
2008
76
female
Nasal, pleura
bilateral
Biopsy of the nasal tumor
Chemotherapy (THP-COP)
(Died) 60 days after admission
No
Nagireddy et al. [ 5]
2011
70
male
Meninges, left middle cranial fossa, paranasal sinus, breast, lungs, pleura, kidney, and prostate
bilateral
CT-guided biopsy
Chemotherapy (CHOP)
(Died) one month later while on hospice
Yes
Kang et al. [ 6]
2011
29
male
Multiple cervical, mediastinal and abdominal lymph nodes
bilateral
Excisional biopsy of cervical lymph nodes
Chemotherapy (ifosfamide, methotrexate, etoposide and prednisolone)
(Died) 59 days after his first admission
No mention
Jin et al. [ 7]
2005
37
male
Oral cavity, pharyngel, spleen, lymph nodes
bilateral
None
Chemotherapy (CHOP)
(Died) 21 days after admission
No mention
Our case
2014
28
male
Kidney
unilateral
Adrenalectomy
Surgery, chemotherapy (CHOP) and radiotherapy
(Alive) 26 months after treatment
Yes
Because of the rarity of this disease, the standard therapies have not been defined. The treatments have included various combinations of surgery, chemotherapy, and radiation. In the reported cases, four patients received chemotherapy, one patient received surgery and chemotherapy; one patient received high dose intravenous dexamethasone, and one patient received immunosuppression therapy. CHOP was the most common used chemotherapy regimen in these reported cases. The longest reported survival was 90 days in a patient who received surgery and chemotherapy. Our patient underwent surgery, postoperative chemotherapy with 4 cycles of CHOP followed by radiotherapy. Two more cycles of CHOP were administered afterwards. It should be noted that the surgery was performed prior to the patient’s visit at our hospital. If a biopsy sufficed, then surgery would not be necessary, given that these tumors are generally sensitive to chemotherapy and radiation therapy. Because of the residual tumor at the left side of the retroperitoneum, postoperative radiation therapy was recommended. In terms of chemotherapy regimen, SMILE (dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide) is a standard chemotherapy regimen for NK/T lymphoma patients in the western countries. However, there was a shortage of L-asparaginase in our province in the end of 2013, when this patient was first treated. Therefore, this patient was treated with CHOP according to the previous case reports [ 2, 5, 7]. As a side note, SMILE has been used for NK/T lymphoma patients in our province since mid-2014.
Our patient remains disease-free with a follow up of over two years. We list three possible reasons for the longer disease-free survival for our patient: (1) the NK/T cell lymphoma only involved one adrenal gland; (2) the NK/T cell lymphoma did not involve other organs, except for the adjacent kidney; and (3) the utilization of trimodality therapy.

Conclusion

In conclusion, primary adrenal NK/T cell lymphoma is an exceedingly uncommon neoplasm and the outcomes for it are generally poor. We present the first case of a primary adrenal NK/T cell lymphoma in China with over 2-year disease-free survival treated with trimodality therapy. NK/T cell lymphoma should be considered in the differential diagnosis of a rapidly enlarging adrenal mass. As reports of such cases increase and our understanding of the disease improves, we anticipate more effective treatment for these patients.

Acknowledgements

None.

Funding

Applied Basic Research Program in Wuhan (Grant No. 2015060101010054); Natural Science Foundation of Hubei (Grant No. 2016CFC737).

Availability of data and materials

All relevant data are within the manuscript.

Authors’ contributions

LH and WMX drafted the manuscript and performed the literature search; MWW carried out pathological examination and contributed to the histopathological details of the manuscript; PW acquired the clinical data and was responsible for interpretation of the data; GH and CL conceived the idea for this case report, referred to the literature review aspect of this case report and revised the article for important intellectual content. All authors were responsible for critical revision of the manuscript. All authors have read and approved the final manuscript.

Authors’ information

Liu Hu, Radiation Oncologist
Weimin Xu, Surgeon
Mingwei Wang, Pathologist
Pan Wang, Physician
Guang Han, Radiation Oncologist
Chi Lin, Radiation Oncologist

Competing interests

The authors declare that they have no competing interests.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editor of this journal.

Ethics approval and consent to participate

Not applicable.

CARE guidelines/methodology

The authors adhered to CARE guidelines/methodology.
Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://​creativecommons.​org/​licenses/​by/​4.​0/​), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://​creativecommons.​org/​publicdomain/​zero/​1.​0/​) applies to the data made available in this article, unless otherwise stated.
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