Adams, Heiner

Hans, Volkmar

Hänggi, Daniel

Probst, Alphonse

Tolnay, Markus

RERO DOC Digital Library

LETTER TO THE EDITORDaniel Ha¨nggi Æ Heiner Adams Æ Volkmar H. HansAlphonse Probst Æ Markus TolnayRecurrent glomus tumor of the sellar region with malignant progressionReceived: 24 January 2005 / Revised: 21 February 2005 / Accepted: 21 February 2005 / Published online: 11 June 2005 Springer-Verlag 2005Glomus tumors are small, benign mesenchymal neo-plasms, the majority of which occur in the dermis orsubcutis of the extremities. The single most common siteis the subungual region of the ﬁngers, followed by palm,wrist, forearm and foot. The tumor cells resemble mod-iﬁed smooth muscle cells of normal glomus bodies.Glomus tumors, however, have been reported in loca-tions in which the glomus bodies are sparse or even ab-sent, among them patella, nerve, eyelid, liver,gastrointestinal tract, trachea, nose, bone and sella tur-cica [3, 9]. Glomus tumors usually show a benign clinicalcourse. However, rare malignant cases have been re-ported [5, 6, 7]. In a recent review on atypical andmalignant glomus tumors, Folpe et al. [3] have proposeda classiﬁcation scheme, encompassing malignant glomustumors, symplastic glomus tumors, glomus tumors ofuncertain malignant potential and glomangiomatosis.Glomus tumors in the sellar region are exceedinglyrare and to the best of our knowledge only one case hasbeen reported so far in this location [2]. Here we reportthe case of an intra- and parasellar neoplasm ﬁrstdiagnosed as a benign glomus tumor. Recurrent tumorgrowth was observed 8 and 10 years after the ﬁrst sur-gical intervention and subsequent histology suggestedmalignant progression of the glomus tumor.In 1991, 9 months after a moderate cervical spinetrauma, the 47-year-old female patient noted intermit-tent diplopia. Ophthalmological examination revealed aright-sided cavernous sinus syndrome with palsy of theocculomotor, trochlear and abducent nerves. Cranialcomputer tomography and magnetic resonance imaging(MRI) showed an intrasellar mass lesion that extendedinto the right-sided supra- and parasellar regionsincluding the cavernous sinus. Microsurgical tumorresection was performed with a right pterional craniot-omy approach. The residual tumor was treated byfractioned radiation therapy (27 fractions with a totaldose of 54 Gy). Apart from a persisting right-sidedtrochlear nerve palsy the clinical history was uneventfultill May 1999, when the patient developed a right-sidedlid ptosis and an intermittent diplopia. On MRI scans,the tumor was found to have recurred and expanded tothe right supra- and parasellar regions, now measuring3 cm in diameter (Fig. 1A). Incomplete tumor resectionwas performed via a transsphenoidal approach followedby gamma knife radiosurgery with a total dose of 25 Gy.In July 2001 the patient noted further visual impairmentand ophthalmological testing revealed loss of visualacuity and visual ﬁeld defects in the right eye. Endocri-nological examination demonstrated panhypopituita-rism. Repeated MRI scans again showed tumor growthin the right supra- and parasellar region measuring up to3.5 cm in diameter. The patient underwent right-sidedpterional recraniotomy with incomplete tumor resection.Due to progressive visual impairment a further trans-sphenoidal tumor resection was undertaken in Novem-ber 2001. Further radiation therapy was initially refused;however, due to progressive tumor growth (Fig. 1B) atargeted radiotherapy using 90Yttrium-DOTATOC, anYttrium-labeled somatostatin analogue, was conductedearly in 2003. The patient died in October 2003, 12 yearsafter initial manifestation of the tumor. No autopsy wasperformed.D. Ha¨nggiDepartment of Neurosurgery,University Hospital Du¨sseldorf,Du¨sseldorf, GermanyH. Adams Æ A. Probst Æ M. Tolnay (&)Department of Neuropathology,University Hospital Basel,Scho¨nbeinstrasse 40, 4003 Basel, SwitzerlandE-mail: mtolnay@uhbs.chTel.: +41-61-2652525Fax: +41-61-2653194V. H. HansHirntumor-Referenzzentrum,University Hospital Bonn,Bonn, GermanyD. Ha¨nggiDepartment of Neurosurgery,University Hospital Basel,Basel, SwitzerlandActa Neuropathol (2005) 110: 93–96DOI 10.1007/s00401-005-1006-8Surgical specimens were all ﬁxed in 10% formalin,routinely processed and embedded in paraﬃn. Five-micrometer thin sections were cut and stained withhematoxylin and eosin, periodic acid-Schiﬀ (PAS) andsilver reticulin stain. Immunohistochemistry was per-formed, following standard protocols using a broadpanel of antibodies (see below). Small tumor fragmentswere cut from paraﬃn-embedded tissue blocks andprocessed for electron microscopy.Histology of the initial tumor revealed a predomi-nantly solid neoplasm composed of sheets of uniformround and polygonal cells interrupted by small thin-walled vessels (Fig. 1C). Vessels were not invaded by thetumor. Tumor cells were of regular shape with a pun-ched-out rounded nucleus, surrounded by clear, slightlyeosinophilic cytoplasm. The nuclei contained ﬁnely dis-persed chromatin with an indistinct nucleolus. Silverreticulin stain revealed a delicate ﬁber network sur-rounding individual tumor cells or small cell groups.Mitoses and necrosis were absent.Fig. 1 A T1-weighted MRI scan of the ﬁrst recurrent tumor in1999, showing predominantly right-sided parasellar contrastenhancing mass with inﬁltration of the cavernous sinus andcompression of the temporal lobe. B T1-weighted MRI scan,performed in early 2003, demonstrating the uniformly enhancingintra-suprasellar mass with direct contact to the optic chiasm. CHematoxylin and eosin staining of the initial tumor. Uniformround cells are arranged in sheets and interrupted by small vessels.D, E The tumor after the second (D) and third (E) recurrence ishypercellular, tumor cells and nuclei are more polymorphous, andseveral mitoses can be seen (arrows). FMIB-1 labeling of the tumorafter third recurrence. G–I Immunostaining of the tumor after theﬁrst recurrence; G smooth muscle actin, H CD99, and I lamininimmunoreactivity. Bars A, B 1 cm; original magniﬁcation C ·200;D, E, G–I ·400; F ·50094The histological appearance of the tumor specimensobtained at the time of the second resection was quitesimilar to that of the ﬁrst biopsy. However, there werefoci of higher cellularity, and tumor cells and the nucleiappeared more polymorphous. One mitosis per 50high-power ﬁelds (HPF) was noted, but there were noatypical mitotic ﬁgures (Fig. 1D). Tumor necrosis wasabsent. Histology from the third and forth biopsies wasfound to be very similar to the second biopsy. In con-trast to the previous tumor specimens, however, therewere more cellular and nuclear atypia. Up to 3 mitoses/50 HPF were noted (Fig. 1E). Again, there was no tu-mor necrosis.Immunohistochemically, tumor cells stained stronglyfor vimentin, smooth muscle actin, laminin and CD99(Fig. 1G–I), and a few cells stained faintly for neuron-speciﬁc enolase and epithelial membrane antigen. CD34only stained the tumor vasculature. The MIB-1 prolif-eration labeling index was below 1% in the initial tu-mor, about 2% in the second tumor specimens and upto 10% in the biopsies taken in 2001 (Fig. 1F). Tumorcells were negative for chromogranin A, S-100 protein,cytokeratin, placental alkaline phosphatase, synapto-physin, glial ﬁbrillary acidic protein, desmin, CD117and pituitary hormones.Electron microscopy revealed medium-sized cellswith interdigitating microvilli on their surface. Thetumor cells possessed equally sized, sometimes slightlygrooved, nuclei with a nucleolus. The most prominentcytoplasmic features consisted of sparse pinocytoticvesicles, large mitochondria and focal aggregates ofmicroﬁlaments. Few individual cells were surroundedby a layer of basal lamina. Cells were arranged ‘backto back’ and were separated by bundles of longspacing collagen.Glomus tumor of the sellar region is rare. To ourknowledge, the case reported by Asa and coworkersmore than 20 years ago [2] is the only known case sofar. We now report histological, immunohistochemicaland electron microscopic ﬁndings of a second case of asellar glomus tumor. Similar to the case reported byAsa et al. [2], recurrent tumor growth was also observedin our patient.Irrespective of their anatomical location glomus tu-mors are usually benign, but rare malignant cases havealso been described [5, 6, 7]. In our case, benign glomustumor was diagnosed in a ﬁrst biopsy. However, in laterbiopsies, hypercellularity, cellular and nuclear atypia,increased mitotic activity and MIB-1 labeling indiceswere noted, suggesting malignant transformation of theglomus tumor.Recently, Folpe et al. [3] proposed a classiﬁcation ofatypical and malignant glomus tumors. Malignantglomus tumors are deﬁned as tumors with: (i) large size(>2 cm) and deep location, (ii) atypical mitotic ﬁgures,or (iii) marked atypia with mitotic activity (e.g., >5mitoses/50 HPF). Symplastic glomus tumors lack cri-teria for malignant glomus tumor and exhibit onlymarked nuclear atypia. Glomus tumors of uncertainmalignant potential are deﬁned as tumors with: (i)superﬁcial location with high mitotic activity, (ii) largesize only, or (iii) deep location only. Finally, glo-mangiomatosis lacks criteria for malignant glomus tu-mor or glomus tumor of uncertain potential, and showsfeatures of diﬀuse angiomatosis and excess glomus cells[3]. In this series, 38% of malignant glomus tumorsmetastasized, but metastatic disease was not seen in anyof the symplastic glomus tumors, glomus tumor ofuncertain malignant potential, or glomangiomatosis.Our case does not fulﬁll the criteria proposed forsymplastic glomus tumors and glomangiomatosis, andis, therefore, very unlikely to correspond to one ofthese entities. There is still the question of whether ourcase does represent a malignant glomus tumor or aglomus tumor with uncertain malignant potential.Atypical mitotic ﬁgures as well as marked atypia withmitotic activity of more than 5 mitoses/50 HPF werenot features in our case. Since the tumor was deﬁnitelylarger than 2 cm and located deep in the sellar region,it would nevertheless qualify for a malignant glomustumor. With regard to the sella turcica, however, thecriteria of ‘‘deep location’’ and ‘‘large size’’ need fur-ther consideration. In their series of unusual glomustumors, Folpe et al. [3] deﬁned the tumor location aseither superﬁcial to the muscular fascia or deep to it.In their study, ﬁve out of nine deeply locatedperipheral soft tissue glomus tumors (all of which werelarger than 2 cm) metastasized. In a series of 32 gas-trointestinal (GI) glomus tumors, there was only 1case that metastasized, although all of these tumors bydeﬁnition were deeply located, and 19 of them werelarger than 2 cm [8]. In another series of gastric glo-mus tumors, none of the 12 tumors metastasized,including those which were larger than 2 cm [1]. Thus,there seems to be a marked diﬀerence in the frequencyof malignant behavior between deep-seated glomustumors of the peripheral soft tissue and those locatedin other ‘‘deep’’ regions. Taking this into account, adeep location as well as a tumor size larger than 2 cmmay not per se be features of malignancy of a glomustumor in the sellar region. Clinical observations over12 years for our patient did not reveal metastases, andthe same is true for the glomus tumor reported by Asaet al. [2], although the follow-up in that case was only6 years. However, both recurrent glomus tumorsshowed a locally inﬁltrative growth pattern into thesupra- and parasellar region. Thus, according to anearlier classiﬁcation proposed by Gould et al. [4], bothcases would represent examples of locally aggressiveand/or potentially malignant glomus tumors, or asglomus tumors of uncertain malignant potentialaccording to the classiﬁcation by Folpe et al. [3]. Suchtumors may recur locally but usually do not metas-tasize [4].In conclusion, our case demonstrates that a glomustumor can occur within the sella turcica, and that it mayrecur locally and invade the supra- and parasellar regionwith an aggressive and inﬁltrative growth.95Acknowledgements We are grateful to Gernot Jundt for usefulcomments on the case and to Michelle Pfeifer for correcting theenglish draft.References1. Appelman HD, Helwig EB (1969) Glomus tumors of the stom-ach. Cancer 23:203–2132. Asa SL, Kovacs K, Horvath E, Ezrin C, Weiss MH (1984) Sellarglomangioma. Ultrastruct Pathol 7:49–543. Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW (2001)Atypical and malignant glomus tumors: analysis of 52 cases,with a proposal for the reclassiﬁcation of glomus tumors. Am JSurg Pathol 25:1–124. Gould EW, Manivel JC, Albores-Saavedra J, Monforte H (1990)Locally inﬁltrative glomus tumors and glomangiosarcomas. Aclinical, ultrastructural, and immunohistochemical study. Can-cer 65:310–3185. Hiruta N, Kameda N, Tokudome T, Tsuchiya K, Nonaka H,Hatori T, Akima M, Miura M (1997) Malignant glomus tumor:a case report and review of the literature. Am J Surg Pathol21:1096–11036. Hishida T, Hasegawa T, Asamura H, Kusumoto M, MaeshimaA, Matsuno Y, Suzuki K, Kondo H, Tsuchiya R (2003)Malignant glomus tumor of the lung. Pathol Int 53:632–6367. Matsumoto K, Kakizaki H, Yagihashi N, Yagihashi S (2001)Malignant glomus tumor in the branchial muscle of a 16-year-old girl. Pathol Int 51:729–7348. Miettinen M, Paal E, Lasota J, Sobin LH (2002) Gastrointesti-nal glomus tumors: a clinicopathologic, immunohistochemical,and molecular genetic study of 32 cases. Am J Surg Pathol26:301–3119. Weiss SW, Goldblum JR (2001) Perivascular Tumors. In: WeissSW, Goldblum JR (eds) Enzinger and Weiss’s soft tissue tumors,4th edn. Mosby, St. Louis, pp 985–103596

Recurrent glomus tumor of the sellar region with malignant progression

http://doc.rero.ch/record/314282/files/401_2005_Article_1006.pdf

Recurrent glomus tumor of the sellar region with malignant progression

Abstract

Similar works

Full text

Available Versions

RERO DOC Digital Library