|Year : 2021 | Volume
| Issue : 1 | Page : 43-46
Langerhans cell histiocytosis of solitary inguinal mass: Diagnosis and treatment of an unusual case with complete response
Sujata Sarkar1, Maitrayee Saha1, Tamohan Chaudhuri1, Shravasti Roy2
1 Department of Radiotherapy, Saroj Gupta Cancer Centre and Research Institute, Kolkata, West Bengal, India
2 Department of Pathology, Saroj Gupta Cancer Centre and Research Institute, Kolkata, West Bengal, India
|Date of Submission||08-Oct-2021|
|Date of Acceptance||16-Nov-2021|
|Date of Web Publication||06-Jan-2022|
Dr. Sujata Sarkar
Batra Housing Complex, Tughlakabad Institutional Area, Mehrauli Badarpur Road, New Delhi 110062.
Source of Support: None, Conflict of Interest: None
Langerhans cell histiocytosis (LCH) is a “histiocytic” disorder with a spectrum of presentations often with different names having confined a bone, overlying skin, and local lymph nodes or having multiorgan involvement. It is characterized by clonal proliferation of Langerhans cells, which have typical grooved nuclei and tennis racquet-shaped Birbeck’s granules. It is more common in children below 10 years. It is more common in men with M:F is 2:1. Bone is the most common site of involvement. Among lymph nodes, cervical chain is most commonly involved and inguinal lymphatic chain involvement is rarely seen. Diagnosis requires histopathological confirmation. Histopathology shows proliferation of dendritic cells expressing high levels of Langerin, a lectin required for the formation of Birbeck granules. It is positive for CD1a, Langerin, S100, CD68, and vimentin. Here, we are reporting a case of LCH of solitary inguinal mass, localized to unilateral pubic bone, overlying skin and inguinal nodes, in a 35-year-old man. Our aim is to discuss about the investigations leading to diagnosis of this rare entity, LCH; its treatment with surgery and adjuvant radiotherapy, which led to complete response and patient is disease free even after 4 years of treatment.
Keywords: Adjuvant radiotherapy, complete response, disease free, Langerhans cell histiocytosis, solitary inguinal mass
|How to cite this article:|
Sarkar S, Saha M, Chaudhuri T, Roy S. Langerhans cell histiocytosis of solitary inguinal mass: Diagnosis and treatment of an unusual case with complete response. Bengal J Cancer 2021;1:43-6
|How to cite this URL:|
Sarkar S, Saha M, Chaudhuri T, Roy S. Langerhans cell histiocytosis of solitary inguinal mass: Diagnosis and treatment of an unusual case with complete response. Bengal J Cancer [serial online] 2021 [cited 2022 May 21];1:43-6. Available from: http://www.bengaljcancer.org/text.asp?2021/1/1/43/335060
| Introduction|| |
Langerhans cell histiocytosis (LCH) is a rare pathology caused by abnormal clonal proliferation of Langerhans cells. It primarily affects children, median age 2–4 years, and is twice more common in men than women. It can be localized to single or multiple sites within a single system or more disseminated and multisystem. Bone is the most commonly involved site. Rarely, LCH can primarily involve lymph nodes without involving other sites, where cervical lymph nodes are the most and inguinal nodes are the least commonly affected nodes. Overall, LCH is very rare with an incidence of 1–4 cases per 10 lakhs population. To the best of our knowledge, in all the case reports on LCH of lymph nodes, it has involved children and with cervical lymph nodes. Here, we are presenting a case of LCH of a solitary inguinal mass, focusing on the investigations done to diagnose a rare malignancy, LCH and treatment done due to which patient is disease free since 4 years.
| Case Report|| |
A 35-year-old man presented in July 2017 with an ulcero-proliferative left inguinal mass since 9 months, which was gradually progressive and painful. The finding of general and systemic examination was unremarkable. No other lymphadenopathy was detected. On local examination, the mass was 3.5 × 2 cm in size, ulcero-proliferative, firm, immobile and tender. Complete blood count, kidney and liver function tests were normal. Mantoux test was unremarkable. CECT neck and thorax was normal. Triphasic computed tomography (CT) scan of the whole abdomen revealed a large enhancing soft tissue mass at the left inguinal region 4 cm × 3 cm involving overlying skin along with left inguinal lymphadenopathy [Figure 1]A.
|Figure 1: (A) Triphasic CT abdomen showing left-sided inguinal mass. (B) Gross photograph of inguinal mass. (C) Photomicrograph showing nodal architecture partially replaced by sheets of small cells. (H&E 4x). (D) Photomicrograph showing grooved Langerhans cell with eosinophils in the background (H&E 40x)|
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Wide local excision of the mass with abdominal flap reconstruction was done. Subtotal resection was done. Residual mass was left over the femoral vessels area; medial extension could not be excised from the pubic bone, that is, R2 resection was done. Intraoperatively, it was observed that the mass penetrated the subcutaneous plane and spread to involve the pubic bone, femoral vessels, inguinal ligament, and spermatic cord.
The gross picture of the mass [Figure 1B] revealed it to be a fleshy, lobulated lesion with overlying ulceration. Histopathology revealed sheets [Figure 1C] of Langerhans cells with abundant clear to eosinophilic cytoplasm and grooved lobulated nuclei, against a background of mixed inflammatory infiltrate [Figure 1D]. There was minimal nuclear atypia and mitotic count of 6 per 10 hpf after extensive sampling. On immunohistochemistry, it was immunoreactive to CD1a, S100, CD68, and vimentin. In our case, specimen was not tested for Langerin due to unavailability in our resources. Cytokeratin (CK), PLAP, RCCMa, LCA, HMB45, and CD99 were negative [Figure 2]. Thus, a final diagnosis of LCH was rendered.
|Figure 2: Photomicrographs of CD1a+, S100+, Vimentin+, CD68+, CD99, and LCA negative in tumor cells (IHC 10x)|
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Four weeks postoperatively, the patient was subjected to adjuvant radiotherapy of dose 44 Gy/22 fractions (Fr) at 1Fr/day and 5 Fr/week in September 2017, by intensity-modulated radiotherapy (IMRT). The patient tolerated radiation well with no complication during or post-radiation. Contrast-enhanced CT (CECT) pelvis was done after 6 weeks which showed complete response. The patient is on regular follow-up since the last 4 years with no complaints.
| Discussion|| |
LCH is one of the “histiocytosis syndromes,” as defined by the Histiocyte Society. LCH is an abnormal proliferation and dissemination of histiocytes. It is named after the appearance of the cells, which resemble the normal dendritic cells first noted by Langerhans. As per WHO, LCH is of three types: LCH with solitary lesion, multiple lesions, and disseminated/with visceral involvement. The Langerhans cells are characteristically immunoreactive to CD1a, CD207 (Langerin), S100, CD68, and vimentin. Electron microscopy shows the pathognomic Birbeck’s granules.
LCH can involve any body tissue, most commonly bone, but it can also involve skin, neurohypophysis, oral cavity, anogenital region, lungs, liver, spleen, and kidney. It was formerly considered as a disease of childhood, but it is often found in adulthood.
There is controversy about whether LCH is a malignancy or an inflammatory lesion. None of the epidemiological studies suggests the infectious or environmental cause. Various previous studies and recent studies by scientists at Dana-Farber Cancer Institute have shown a mutant oncogene, BRAF V600E, in more than half of LCH biopsy samples over time, suggesting LCH as a neoplatic disorder. Also, clonal nature of LH cells and shared mutations with hemopoietic precursors favor the reclassification of LCH as a myeloid neoplastic disorder. BRAF V600E mutations are found in 65%–75% of LCH cases. It activates the MAP-Kinase pathway for cell proliferation. Peripheral blood mononuclear cells (PBMC) express BRAF 600E mutations in high-risk (multiorgan), LCH which is absent in low-risk LCH confined to local skin, bone, and lymph nodes. In high-risk cases, the histiocytes (dendritic cells) are derived from the PBMC or from myeloid progenitor cells in the bone marrow. They migrate into the tissues. The role of MAPK inhibitors (Vemurafenib and Dabrafenib) in high-risk lesions is under clinical trial.
Most patients with LCH will survive this disease. Treatment of the generalized form of LCH involves radiotherapy in combination with chemotherapy, whereas the localized form is treated with surgery with adjuvant radiation. However, due to rare incidence of LCH, there are no randomized trials to show the standard treatment of histiocytosis. Clinical studies showed high radiosensitivity in LCH lesions and a favorable tumor response to low doses of radiation. However, optimal irradiation doses have not been determined yet. In the available publications and various retrospective analysis, radiation treatment was carried out with a median total dose of 15 Gy (range 3–50.4 Gy). The median single fraction was 2 Gy (range 1.8–3 Gy). Patients were treated with Cobalt-60 gamma rays or 5–15 MV photons of a linear accelerator.,, Patients with LCH should have long-term follow-up to detect late complications of the disease or treatment like problems of skeletal deformity or function, liver or lung problems, endocrine abnormalities, dental issues, or neurological and neurocognitive dysfunction. LCH has a long-term control rate of 80% and overall 5-year survival is 90%.
| Conclusion|| |
LCH is overall a rare entity, presenting with non-specific symptoms. Diagnosis requires histopathological confirmation. Further large-scale randomized trials are required to establish standard treatment protocols as it shows good response and long-term survival, especially in the case of solitary lesions.
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]