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Langerhans' Cell Histiocytosis: Possible Association with Malignant Germ Cell Tumour

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Langerhans' Cell Histiocytosis: Possible Association with Malignant Germ Cell Tumour _J Clin Pathol 1995;48:963-965 963 Langerhans' cell histiocytosis: possible association with malignant germ cell tumour J Clin Pathol: first published as 10.1136/jcp.48.10.963 on 1 October 1995. Downloaded from W K Ng, K Y Lam, I 0 L Ng Abstract sometimes occur in association with various A rare case ofadult onset Langerhans' cell types of malignant neoplasms.' Several case histiocytosis associated with dysgermin- reports have been published concerning the oma in a 35 year old Chinese woman is possible association between Langerhans' cell reported. The patient had a history ofdys- histiocytosis and malignant lymphoma, espe- germinoma of left ovary 15 years pre- cially Hodgkin's disease.2" de Camargo et al' viously and had undergone surgery also described the coexistence ofosteosarcoma, followed by radiotherapy and an un- lymphoma, or medulloblastoma and Lan- eventful recovery. She presented again in gerhans' cell histiocytosis in the same patient. March 1994, this time with a left clavicular Malignant germ cell tumour (MGCT) is as- mass, which was shown histologically to be sociated with haematological neoplasia, mainly Langerhans' cell histiocytosis. The report leukaemia.i' However, Langerhans' cell histio- Departnent of illustrates the probable association be- cytosis was not known to associate with Pathology, tween the two lesions, with some dis- MGCT, until a recent case report by Mandel University of Hong et describing a family with clustering of Kong, cussion on the underlying pathogenesis. al,10 Queen Mary (J Clin Pathol 1995;48:963-965) MGCT and Langerhans' cell histiocytosis. We Compound, report a rare case of dysgerminoma and Lan- Pokfulam Road, Keywords: Langerhans' cell histiocytosis, malignant gerhans' cell histiocytosis in a Chinese woman, Hong Kong germ cell tumour. W K Ng which may give some clues to the possible K Y Lam association between the two disease entities. I 0 L Ng Langerhans' cell histiocytosis, previously Correspondence to: Dr W K Ng. known as histiocytosis X, consists of a group Case report Accepted for publication of clinicopathological disorders characterised A 35 year old Chinese woman first presented 13 March 1995 by proliferation of Langerhans' cells. It may in 1979 with a left ovarian mass. Laparotomy revealed a dysgerminoma of the left ovary with metastasis involving the rectal wall. Total ab- http://jcp.bmj.com/ dominal hysterectomy and bilateral sal- pingoophorectomy, together with partial resection ofthe rectum, were performed. There was no clinical evidence of other distant metastases. She was then given postoperative radiotherapy for one year. No adjuvant chemo- therapy was given. on September 28, 2021 by guest. Protected copyright. The postoperative course was uneventful until March 1994 when she noticed pain and swelling over left clavicle for two months. On x ray examination, a 1'5 cm diameter osteolytic lesion was observed in the left clavicle. The clinical diagnosis at that time was metastatic dysgerminoma. Curettage of the lesion was carried out, yielding several small fragments of brownish tissue. The specimen was promptly fixed in 10% neutral formalin and embedded in paraffin wax. Sections, 3 gm thick, were cut and stained with haematoxylin and eosin. The sections were then stained with a polyclonal antibody (diluted 1 in 200; Dako, Copenhagen, Denmark) directed against S- 100 protein using the Streptavidin-biotin complex technique. A representative area was also selected from the paraffin wax block, dewaxed, rehydrated, post- fixed in 1% osmium tetroxide, embedded in epoxy resin, sectioned, and stained with uranyl acetate-lead citrate for subsequent ultra- structural study. Figure 1 Histological section of the left clavicular bony curettage showing Langerhans' cell histiocytosis. Note the characteristic folded nuclei of Langerhans' cells. (Haematoxylin Histological examination ofthe left clavicular and eosin; x 500.) lesion revealed aggregates of ovoid cells ad- 964 Ng, Lam, Ng in the same lymph nodes. One possible ex- planation is that the lymphoma cells may pro- vide a direct or indirect stimulus for the proliferation of these Langerhans' cells. J Clin Pathol: first published as 10.1136/jcp.48.10.963 on 1 October 1995. Downloaded from MGCT, as the name implies, is a malignancy arising from germ cells which may have the potential to differentiate along different lines including yolk sac, trophoblasts and embryonic tissue. Occasional cases of mediastinal, non- seminomatous MGCT associated with haema- tological malignancies have been reported."O Three hypotheses have been put forward67" : (1) leukaemic differentiation of the malignant germ cells; (2) induction of leukaemia by an unknown factor secreted by the germ cell tumour; and (3) the leukaemia and the germ cell tumour may arise independently of each other. In 1989 Chaganti et al8 described a case of mediastinal yolk sac tumour with later leukaemic differentiation, as both tumours showed identical cytogenetic changes. Their results were in agreement with the first hy- pothesis. Mediastinal yolk sac tumour may also be associated with malignant histiocytosis, which may be related to the secretion ofmacro- phage colony stimulating factor or a similar substance by the yolk sac elements.'4 However, there is no previous reported case in the English Figure 2 Electron micrograph of a Langerhans' cell showing characteristic literature mentioning the occurrence of Lan- intracytoplasmic Birbeck's granules in the perinuclear region. Note the barely discernible gerhans' cell histiocytosis and MGCT in the "zip-like" periodic core of the granules with terminal expansion. (Uranyl acetate-lead citrate; x 40 000.) same patient. Our case represents a rare case documenting an occurrence ofadult onset Lan- gerhans' cell histiocytosis 15 years after the diagnosis of dysgerminoma. mixed with small lymphocytes and occasional In 1994 Mandel et all' described a family eosinophils. The ovoid cells had deeply folded with MGCT and Langerhans' cell histiocytosis nuclei with a characteristic "coffee bean" ap- occurring in different family members. The pearance, fine chromatin, inconspicuous nuc- patient with Langerhans' cell histiocytosis leoli, eosinophilic cytoplasm, and ill-defined shared an identical haplotype with her two http://jcp.bmj.com/ cell borders (fig 1). Many of these cells sisters who had a yolk sac tumour and dys- expressed S-100 protein. Ultrastructural germinoma, respectively. Their postulation of examination revealed the presence of in- an association between Langerhans' cell histio- tracytoplasmic Birbeck's granules in the peri- cytosis and MGCT is in agreement with our nuclear region of some of the Langerhans' observation. It is possible that Langerhans' cells (fig 2). The overall features were those of cell histiocytosis, like leukaemia, can share a Langerhans' cell histiocytosis. Bone scan and common stem cell with MGCT; or else the on September 28, 2021 by guest. Protected copyright. skeletal x ray showed no other bony lesions histiocytosis may be the result of stimulation and there was no other evidence of systemic by a "factor" secreted by an occult focus of involvement by Langerhans' cell histiocytosis. residual dysgerminoma. Alternatively, the Lan- The peripheral blood examination was un- gerhans' cell and germ cell proliferations may remarkable. There was no clinical evidence of be triggered by a common stimulus that acts recurrence of dysgerminoma. on both types of cells. The occurrence of the two may also be coincidental. Detailed cyto- genetic and molecular studies in the future Discussion may help to clarify the underlying pathogenic Langerhans' cell histiocytosis is now considered relationship. by some authors to be a probable non-neo- plastic reactive proliferation of Langerhans' 1 Egeler RM, Neglia JP, Puccetti DM, Brennan CA, Nesbit ME. Association of Langerhans cell histiocytosis with cells." 12 The nature of the underlying stimuli malignant neoplasms. Cancer 1993;71:865-73. is not known, though immune dysfunction and 2 Kjeldsberg CR, Kim H. Eosinophilic granuloma as an in- cidental finding in malignant lymphoma. Arch Pathol Lab viral infections have been postulated." The Med 1980;104:37-40. diagnosis of Langerhans' cell histiocytosis is 3 Burns BF, Colby TV, Dorfman RF. Langerhans' cell gran- ulomatosis (histiocytosis X) associated with malignant based mainly on the characteristic cellular lymphomas. Am J Surg Pathol 1983;7:529-33. morphology, appearances on immunohisto- 4 Segal GH, Mesa MV, Fishleder AJ, Stoler MH, Weick JK, Lichtin AE, et al. Precursor Langerhans cell histiocytosis. chemistry, and the ultrastructural identification An unusual histiocytic proliferation in a patient with per- of Birbeck's granules in the cytoplasm. Oc- sistent non-Hodgkin lymphoma and terminal acute mono- cytic leukemia. Cancer 1992;70:547-53. casional cases of Langerhans' cell histiocytosis 5 de Camargo B, Alves AC, Gorender EF, Bianchi A. As- associated with malignant lymphoma, espe- sociation ofmalignancy and Langerhans' cell histiocytosis. Report of three cases. Med Pediatr Oncol 1993;21:451-3. cially Hodgkin's disease, have been 6 DeMent SH, Eggleston JC, Spivak JL. Association between reported,2A mainly coexisting with lymphoma mediastinal germ cell tumors and hematologic malig- Possible association between Langerhans' cell histiocytosis and germ cell tumour 965 nancies. Report of two cases and review of the literature. Rechavi G. Familial clustering of malignant germ cell Am 7 Surg Pathol 1985;9:23-30. tumors and Langerhans'
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