Malignant fibrous histiocytoma of the vulva: a case report | SpringerLink

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Malignant fibrous histiocytoma occurs infrequently on the vulva but nonetheless is the second most frequent sarcoma of this region. The purpose of this report is ...

Arch Gynecol Obstet (2005) 273: 122–125 DOI 10.1007/s00404-005-0047-5


Birol Vural Æ Sebiha O¨zkan Æ Ku¨rsßat Yıldız Aydın C¸orakc¸ı Æ Yesßim Gu¨rbu¨z

Malignant fibrous histiocytoma of the vulva: a case report

Received: 10 February 2005 / Accepted: 24 March 2005 / Published online: 30 July 2005  Springer-Verlag 2005

Abstract Background: Primary sarcomas of the vulva are rare tumors that account for 1.8–3% of all vulvar malignancies. Malignant fibrous histiocytoma occurs infrequently on the vulva but nonetheless is the second most frequent sarcoma of this region. The purpose of this report is to review the diagnosis and therapy of this exceedingly rare tumor. Case: A 72-year-old woman was presented with a vulvar mass that was ultimately found to be a vulvar malignant fibrous histiocytoma. After surgical excision and 9 months of initial diagnosis, she returned with rapid localized progression and pulmonary metastases. Conclusion: Malignant fibrous histiocytoma arising in the vulva represents an example of vulvar sarcomas. Since the diagnostic criteria and natural history of this tumor still remain poorly defined, further case studies may be helpful to elucidate these issues as well as the optimal therapeutic approach of this heterogeneous group of malignancies. Keywords Malignant Æ Fibrous Æ Histiocytoma Æ Vulvar sarcoma

Introduction Primary sarcomas of the vulva appear as extremely rare cases. Of those, leiomyosarcoma is the most frequently encountered histopathologic variant [1]. On the other hand malignant fibrous histiocytoma (MFH) which is characteristically a tumor of late adult life, may arise in B. Vural Æ S. O¨zkan (&) Æ A. C¸orakc¸ı Department of Obstetrics and Gynecology, At Kocaeli University School of Medicine, Tu¨pra+ Sitesi, Gu¨ney Mahallesi, 7. Sokak, No.: 20, Kat:2, Ko¨rfez, Kocaeli, Turkey E-mail: [email protected] K. Yıldız Æ Y. Gu¨rbu¨z Department of Pathology, At Kocaeli University School of Medicine

the vulva infrequently [2]. It usually presents as a large solitary mass in middle aged women [3]. Since the natural progression of the tumor is still poorly defined, an effort has been made to assign the accurate histopathologic diagnosis and to elucidate the response to therapy and clinical outcome of this heterogenous group of malignancies [4]. A rare case of vulvar MFH is presented herein with review of literature.

Case A 72-year-old woman, gravida 4 para 4 was presented with a 2 months history of a tender pruritic lesion of the vulva. The initial biopsy was reported as vulvar carcinoma, and she was subsequently referred to the university clinic. Her gynecologic examination revealed a verrucous mass lesion originating from the medial site of the left labium minus and clitoris measuring 6·4 cm in size. There was another nodular mass at the left labium majus, initially thought to be a Bartholin cyst, and another discolored lesion involving the right labium. No lymphadenopathy was palpated. Gynecologic ultrasonographic scan did not demonstrate abnormal findings. Laboratory evaluation and tumor markers (Ca 12.5: 13.2 U/ml, Ca 15.3: 10.4 U/ml, Ca 19.9: 4.64 U/ml, AFP: 1.48 U/ml, CEA: 1.65 ng/ml) appeared in the normal range. She underwent a subsequent magnetic resonance imaging study, which revealed a vulvar hyperintense well-circumscribed mass lesion 6·4 cm in size. Chest X-ray found no pathology. On March 27th 2002, excisional biopsies of the verrucous lesion, nodular mass and discoloured lesion localized on the vulva and cystoscopy were performed under general anesthesia. Cystoscopic examination was shown to be normal. The nodular mass diagnosed at the left labium majus and the discoloured lesion located at the right labium majus were reported to be Bartholin cyst and seborrheic keratosis, respectively. On the other hand histopathologic examination of the verrucous le-


sion excised with additional peripheral healthy tissue 1 cm in size was reported as MFH. Adipose tissue peripheral to the primary mass lesion was demonstrated to be free of tumor. The macroscopic appearance of the excised lesion consisted of an ellipse of vulvar skin attached to subcutaneous tissue. Macroscopically, the 6·4 cm lesion was verrucous in appearance, well demarcated from the surrounding tissue, and was gray–white in color. Microscopic appearances of MFH arising from vulva are demonstrated in Figs. 1 and 2. Microscopic examination revealed a diffuse tumor infiltration consisting of predominantly spindle cells arranged in short fascicles intersecting each other. Pleomorphism and mitotic multinucleated activity were prominent. There were scattered multiucleated giant cells besides foci in storiform pattern. The MFH is essentially a diagnosis of exclusion. Before the final diagnosis it is necessary to exclude a number of similar appearing spindle cell lesions such as spindle cell carcinoma, leiomyosarcoma and peripheral nerve sheath tumor. This usually requires the use of special studies, typically immunohistochemical stain. Immunohistochemical analysis demonstrated diffuse strong positivity for CD68 and vimentin, which are used as histiocytic and mesenchymal markers, respectively. Smooth muscle actin and desmin, CD34 (a vascular marker), HMB45 (malignant melanoma marker) and pancytokeratin (an epithelial marker), S100 (to exclude malignant peripheral nerve sheath tumor) expressions were absent. Finally histopathologic diagnosis was MFH, storiform–pleomorphic type. Since the periphery of the tumor was free of tumor with no involvement of the fascia and similar results of radical vulvectomy versus wide local excision were observed, the latter procedure was decided to be the optimal therapy. Subsequently the patient was Fig. 1 Short fascicular tumor cells forming storiform pattern are demonstrated. There is slight pleomorphism (Hematoxylene—Eosine ten times)

discharged to be followed up. Unfortunately she was admitted to hospital with pulmonary metastasis and inguinal lymphadenopathy after 9 months of the diagnosis. Biopsy of the inguinal lymphadenopathy confirmed the initial diagnosis. Control Magnetic Resonance Imaging of lungs demonstrated multiple hyperdense nodular lesions and right paratracheal lymphadenopathy which were supposed to be pulmonary metastatic lesions. Additional radiotherapy was indicated but the patient did not accept the therapy.

Discussion Sarcomas of the vulva are infrequent neoplasms representing 1–3% of vulvar malignancies. Differential diagnosis of the sarcomas includes leiomyosarcoma, neurofibrosarcoma, reticulosarcoma, MFH, angiosarcoma, liposarcoma and lymhoma [5]. The MFH is characteristically a neoplasm of late adult life with the majority of cases between the ages of 50–70 years [5, 6]. It is usually encountered as deep soft tisse or skeletal muscle tumor localized on the extremities or as a retroperitoneal tumor. Additionally unusual localizations such as renal pelvis, cranium, lungs, spleen, colon, ileum, vagina, and vulva are involved [5, 7]. The MFH of the female genital tract is extremely rare [6, 8]. Clinical review of a few number of these cases is demonstrated in Table 1. Recent studies suggest that MFH arises from undifferianted mesenchymal cells, and is typically a solid tumor characterized by infiltrative growth and nuclear polymorphism with giant cells and multinucleated cells. The tumor manifests a broad range of histologic appearance such as storiform-pleomorphic, myxoid, giant cell, inflammatory [2, 3]. It is capable of hema-

124 Fig. 2 Tumor cells showing storiform pattern under high power magnification. A few lymphocytes are seen between tumor cells (Hematoxylene—Eosine 40 times)

togenous and lymphatic metastasis. The clinical followup appears to be associated with a 2 years survival of 60% and a recurrence rate of 44% [9]. Additionally further evidence of ultrastructure and immunohistochemistry may establish the diagnosis. The MFH cells containing a-1 antichymotrypsin and/or a-1 antitrypsin can be identified in approximately in 80% of the cases. Although several workers earlier suggested that the presence of these two histiocytic enzymes, a-1 antitrypsin and a-1 antichymotrypsin within these tumors supported their histiocytic origin, it has been shown that they can be present within sarcomas of diverse types as well as carcinomas possibly as a result of endocytosis from plasma. Fibroblast associated antigen

could be identified on the surface of tumor cells and laminin intracytoplasmically. Additionally mutant P53 could be detected immunocytochemically in about 30% of the cases although the significance in terms of prognosis is not clear [3]. In our case, smooth muscle actin, desmin CD34, HMB45, and pancytokeratin expressions were absent while strong positivity for CD68 and vimentin (histiocytic and mesenchymal markers, respectively) was demonstrated. Characteristically MFH localized at the genital tract is locally invasive and involvement of the underlying fascia increases the risk of local or distant metastasis. The primary therapy is wide local excision or radical vulvectomy. Metastasis of MFH correlates best with the

Table 1 Clinical review of malignant fibrous histiocytoma cases originating from female genital tract Author



Clinical outcome

Hensley and Friedrich [7], Am J Obstet Gynecol


Radical vulvectomy +Bilateral groin dissection +Pelvic lymhadenectomy

Webb et al. [3], Am J Obstet Gynecol


Wide local excision

Davos and Abell [9], Gynecol Oncol

Vulva (two cases) Vulva

Wide local excision

Died of recurrent disease after 11 months of diagnosis, pleural, peritoneal implants, metastasis to cardiac ventricules Was free of recurrent disease after 16 months of excision No adjunctive therapy

Taylor et al. [8], Obstet and Gynecol Malfetano et al. (1986), Br J Obstet Gynecol


Santala et al. [4], Gynecol Oncol


Elchalal et al. [5], Gynecol Oncol


Grisaru et al. [6], Eur J Obstet Gynecol Reprod Biol


Right hemivulvectomy +Unilateral groin dissection Wide local excision Radical vulvectomy with node dissection Radical vulvectomy with node dissection Wide local excision

No adjunctive therapy No adjunctive therapy, was free of recurrent disease after 1 year of excision No adjunctive therapy Combined chemotherapy and radiotherapy complete tumor regression with no evidence of metastasis after 6 years of therapy No adjunctive therapy, was free of disease after 5 years of follow-up


depth of invasion of the original tumor [3, 4]. Lymphadenectomy is reserved for those cases with clinical evidence of regional involvement. Postoperative radiotherapy or chemotherapy are considered as additional therapies in advanced cases or in patients in whom complex medical problems preclude extensive surgical procedures [9]. The number of cases is insufficient to permit a definitive state about the prognostic outcome but some of the patients reported have had recurrence or died of metastasis [3]. Since the tumor was localized, and not invasive to underlying fascia and peripheral tissue was free of tumor in our case and clinical results of radical vulvectomy and wide local excision seem to be similar, we preferred wide local excision without adjunctive therapy. After 9 months of diagnosis, MR imaging demonstrated multiple pulmonary metastatic lesions involving both lungs unfortunately and clinical inguinal lymphadenopathy was noted that was absent initially. The patient refused to have additional radiotherapy. As a conclusion, these tumors are usually diagnosed following surgical excision of the mass. Close observation and excisional biopsy of potentially suspicious lesions are mandatory to elucidate the pathophysiology of this infrequent vulvar malignancy. Since literature presents an insufficient number of cases described in the female genital tract, experience is limited to permit a definitive statement about alternative therapeutic approaches and prognosis of these cases. We presented an uncommon case of vulvar MFH of which the peripheral tissues were free of tumor histopathologically, no adjunctive therapy was offered and unfortunately the

patient was demonstrated to have multiple pulmonary metastatic lesions after 9 months of diagnosis with an absolutely agressive progression of the initial tumor. Further experience is required to assign the most accurate and appropriate diagnosis and therapeutic approach in management of these infrequent tumors.

References 1. Weiss SW, Goldblum TR (2001) Malignant fibriocytocytic tumors. Enzinger and Weiss’s soft tissue tumors, 4th edn. A Harcourt Health Sciences Company, Mosby, 11830 Westline Industries, Drive St Louis, Missouri 63146 2. Kurman RJ (2002) Premalignant and malignant tumors of the vulva. Blaustein’s pathology of the female genital tract, 5th edn. Springer-Verlag, New York 3. Webb MJ, Symmonds RE, Weiland LH (1974) Malignant fibrous histiocytoma of the vagina. Am Obstet Gynecol 119:190 4. Santala M, Sounio S, Syrjanen K, Uronen MT, Saarikoski S (1987) Malignant fibrous histiocytoma of the vulva. Gynecol Oncol 27(1):121–126 5. Elchalal U, Dgani R, Zosmer A, Levi E, Rakovsky E, LifschitzMercer B (1991) Malignant fibrous histiocytoma of the vagina and vulva successfully treated by combined chemotherapy and radiotherapy. Gynecol Oncol 42(1):91–93 6. Grisaru D, Peyser MR,Bernstein-Lipschitz L, Yaron Y, Lessing JB (1998) Malignant fibrous histiocytoma with myofibroblastic differiantion of the vulva in an adolescent female. Eur J Obstet Gynecol Reprod Biol 79(2):219–221 7. Hensley GT, Friedrich EG (1973) Malignant fibroxanthoma: a sarcoma of the vulva. Am J Obstet Gynecol 116:289 8. Taylor RN, Bottles K, Miller T, Braga C (1985) Malignant fibrous histiocytoma of the vulva. Obstet Gynecol 66:145 9. Davos I, Abell MR (1976) Soft tissue sarcomas of the vulva. Gynecol Oncol 4:70

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