Journal of Clinical Pathology, 1979, 32, 616-624 J Clin Pathol: first published as 10.1136/jcp.32.6.616 on 1 June 1979. Downloaded from Female adnexal tumour of probable Wolffian origin Y. SIVATHONDAN, R. SALMI, P. E. HUGHESDON, AND J. M. FACCIN12 From the Departments of Morbid Anatomy of University College Hospital Medical School, London and the Royal Cornwall Hospital (Treliske), Truro, Cornwall, UK SUMMARY Two further cases of 'female adnexal tumour of probable Wolffian origin' are described Both were retroperitoneal and presented a unique histology of uniformly close-packed gland spaces. solid islands, cords, and diffuse areas. A small hamartoma in a female fetus, part of which resembled the tumours, was traced to an area of near apposition with some paroophoron canals, providing further evidence of a Wolffian origin. The literature of this and some other putative Wolffian tumours is briefly reviewed, and the relation to rete adenomas is discussed. True tumours of the broad ligament are uncommon fibroids and a grapefruit-sized tumour extending and many of them resemble primary tumours of the from the lateral pole of the right ovary between the ovary and uterus (Gardner et al., 1957). Recently, layers of the broad ligament; this was removed Karaminejad and Scully (1973) described nine together with the uterus and adnexa. The nature of cases of a rare broad ligament adenoma for which the tumour was unclear until after publication of the they proposed an origin from the Wolffian (meso- article cited when Dr Scully, to whom sections hadcopyright. nephric) duct and tubules, on account of its site and been sent, kindly confirmed the diagnosis. At the histological distinctiveness, even though it bore no time diagnoses of 'arrhenoblastoma of intermediate resemblance to any known mesonephric derivative. type' and 'adenomatous granulosa cell tumour' were The chequered history of the term 'mesonephroma' entertained but considered to be unsatisfactory. The led them to prefer the title 'female adnexal tumor of patient was given a course of postoperative radio- The article de- therapy (3000r to the pelvis and 2600r to the abdo- probable Wolffian origin'. present http://jcp.bmj.com/ scribes two more such cases and supplies further men) and in 1976 was free from recurrence and well evidence from fetal material to support a meso- apart from intercurrent illness (ischaemic heart nephric origin. disease). Case reports CASE 2 Mrs R. aged 62, para 2, 10 years past the menopause, CASE 1 was admitted to hospital with a history of inter- Mrs F. aged 52, para 0, one year past the menopause, mittent postmenopausal bleeding. She had had no on September 30, 2021 by guest. Protected was admitted to hospital with frequency of mic- relevant past illnesses. Two previous curettages had turition of some months' duration. Her past history shown retrogressed cystic endometrial hyperplasia was unremarkable save for mild hypertension, and with polypi, and, as the uterus was enlarged, she had no signs of virilism. A mass was felt rising hysterectomy was recommended. Operation (10 June out of the pelvis, and operation (10 December, 1957; 1975; Mr Brian Cox) disclosed a uterus enlarged by Mr Herbert Reiss) disclosed a few small uterine fibroids and fixed by adhesions, due possibly to diverticulitis, and a retroperitoneal mass adjacent to 'Present address: Department of Histopathology, Royal the left ovary. Panhysterectomy was performed, with Postgraduate Medical School, Hammersmith Hospital, dissection of the mass by medial reflection of the Ducane Road, London W12 colon, together with removal of a small calcified 2Present address: Director of Pathology, Centre de fibroid behind the bladder. The nature of the retro- Recherche Laboratoires, Pfizer, Amboise, France peritoneal tumour was unclear, diagnoses of 'malignant synovioma' and 'metastatic adeno- Received for publication 4 December 1978 carcinoma' being entertained but considered to be 616 J Clin Pathol: first published as 10.1136/jcp.32.6.616 on 1 June 1979. Downloaded from Female adnexal tumour of probable Wolffian origin 617 unsatisfactory; it was finally identified by comparison Microscopic pathology with that of case 1. The patient was well and free from recurrence in May 1977. Both tumours resembled those described by Kara- minejad and Scully (1973) in consisting of winding Macroscopic pathology and branching tubules of varied size (Fig. 1), mostly small; solid islands and cords, also variable; and CASE 1 areas of diffuse growth, closely packed into a massive, The uterus, 9 x 9 x 4 cm, contained a few fibroids, non-lobular architecture with scanty, often barely the largest 3 cm in diameter. The tubes were unre- perceptible stroma. There were, however, some markable, and the left ovary, 3 x 1 5 x 0-8 cm, was differences between the two cases. small and firm. The right ovary spread out at its In case 1 the cells were conspicuously dark, with lateral pole to form a surface plaque on an irregu- scanty cytoplasm and nondescript nuclei, mostly larly ovoid tumour, 15 x 12 x 8 cm, which else- ovoid or distorted into an oblong or pear shape where had a thin capsule and on section was soft, (Fig. 2). Those lining the tubules were cubical, solid, lobulated, and butter-yellow. sometimes flattened in dilated forms, and pseudo- stratified in smaller ones. Many larger tubules had a CASE 2 leafy, indented outline, while the smaller were mostly The uterus, 12 x 6 x 6 cm, showed multiple fibroids, round, ovoid, or reniform, recalling a tubular the largest 4 cm in diameter, an endometrial polypus, adenoma. Cords varied from 1 to 4 cells in thickness; and a cervical mucous polypus. The ovaries were most were two-celled with their cells orientated along slightly enlarged, firm and yellow. The tumour, 10 x the line of the cords or obliquely to them rather than 8 x 5-5 cm, was encapsulated, firm and rubbery, transversely. Diffuse areas acquired a sarcomatoid and pale brown on section, with a few small cavities. appearance (Fig. 3) with interlacing and radiate copyright. http://jcp.bmj.com/ on September 30, 2021 by guest. Protected Fig. 1 Case 1. Low-power view showing massive, close-packed arrangement of interwoven tubules with occasional dilatation and no lobular subdivision. Haematoxylin and eosin x 50 J Clin Pathol: first published as 10.1136/jcp.32.6.616 on 1 June 1979. Downloaded from Fig. 2 Case 1. The same section as in Fig. 1 at higher power showing packed variegated tubules with dark cubical epithelium. H and E x 125 copyright. http://jcp.bmj.com/ on September 30, 2021 by guest. Protected Fig. 3 Case 1. Diffuse sarcomatoid area with ill-formed acini and cords on the left. H and E x 125 Female adnexal tumour ofprobable Wolffian origin 619 J Clin Pathol: first published as 10.1136/jcp.32.6.616 on 1 June 1979. Downloaded from E ;X.N _ _ Ni- - _MU. J,WA _. c /_ Fig. 4 Case 1. Reticulum impregnation showing mainly peri-insular, occasionally pericellular, copyright. arrangements. Gordon and Sweet x 125 patterns of fusiform cells and occasional cord-like absent. The ovaries in case 2 showed marked stromal condensations. The reticulin pattern was mostly hyperplasia with foci of thecosis, and the endo- peri-insular, bringing out the essentially alveolar metrium cystic hyperplasia, probably in early retro- pattern of the tumour even in diffuse areas, but gression but with active areas, a large endometrial http://jcp.bmj.com/ occasionally in the latter it was pericellular (Fig. 4). polypus, and occasional mitoses. No elastic tissue was found and no smooth muscle Both tumours showed occasional papillary pro- was certainly identifiable by PTAH staining. Some trusions into dilated tubules, not convincingly lumina, especially in dilated tubules, contained a glomeruloid (Fig. 6), and both showed a PAS- few exfoliated cells and a little PAS-positive mat- positive basement membrane around tubules and erial. Mitoses were present but scanty. The left ovary cords (Fig. 7), reinforcing the alveolar impression of was atrophic, and the endometrium showed an the reticulum. Both showed a few, small, irregular on September 30, 2021 by guest. Protected active cystic hyperplasia. areas of poorly cellular connective tissue, which In case 2 the cells had more cytoplasm, which was nowhere extended to divide the massive continuum pale or weakly eosinophilic, and sharply defined into lobules, and both showed at low power an ovoid vesicular nuclei. Those lining the tubules were interlacing, rather divergent growth habit: a capsule mostly columnar, with pseudostratified nuclei; a few of poorly cellular connective tissue in which narrow gave the impression of ciliation. Lumina were gen- cords of tumour cells extended in places to the outer erally empty but occasionally contained a little PAS- surface. positive material. There were many clusters of The ovaries, broad ligaments, and tubes of a dilated tubules with low or flat epithelium, producing 26-week-old stillborn female infant (case 3) were a sieve-like, angiomatoid appearance (Fig. 5). In the reduced to interrupted serial sections. At one point solid cords, the cells and nuclei remained epithelioid in the mesosalpinx was a microscopic hamartoma, and similar, with random orientation. Diffuse areas about 0.5 mm maximum diameter, consisting of an were inconspicuous and consisted chiefly of closely epidermoid formation continuous with a cluster of packed cords, but a mesenchyme-like appearance was small tubules and solid islands (Fig. 8), recalling assumed in a few subcapsular areas. Mitoses were those in the case 1 tumour; the resemblance was 620 Y. Sivathondan, R. Salm, P. E. Hughesdon, and J. M. Faccini J Clin Pathol: first published as 10.1136/jcp.32.6.616 on 1 June 1979. Downloaded from copyright. Fig. 5 Case 2. Mesothelioid area showing tubules lined by low or flat epithelium with some intervening cords.