Clinical Thyroidology / Original Paper. Eur Thyroid J 2014;3: DOI: /

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1 Clinicl Thyroidology / Originl Pper Received: December 16, 2013 Accepted fter revision: My 26, 2014 Published online: August 9, 2014 Mysthenic Crisis Mnifesting s Postopertive Respirtory Filure following Resection of Unsuspected Intrthorcic Thymic T-Cell Lymphom during Thyroidectomy for n Adjcent Lrge Retrosternl Goiter Mohmed E. Ahmed, b Mohmed A. Mhgoub Mohmed G. Alnedr Seif I. Mhdi, b Mh Alzubeir c Lmy A.M. El Hssn e ElWleed M. Elmin f Ahmed Mohmmed El Hssn d Khrtoum Teching Hospitl, nd Deprtments of b Medicine nd c Surgery, Fculty of Medicine, t d University of Khrtoum, e University of Ahfd, nd f Alzeim Alzhri University, Khrtoum, Sudn Wht Is Known bout This Topic There hs been one previous cse report on the coexistence of thymom nd intrthorcic goiter with n uncomplicted postopertive course. Mysthenic crisis occurs postopertively in one third of thymom ptients. Wht This Cse Report Adds The presence of thymom in this ptient s lrge intrthorcic goiter ws recognized only postopertively upon the development of mysthenic crisis. Preopertive symptoms of ftigue nd dysphgi nd ppliction of predictive score for mysthenic crisis might hve lerted the treting physicin t n erlier stge. Key Words Thymom Goiter Medistinl neoplsms Substernl goiter Mystheni crisis Abstrct A middle-ged femle with goiter of 10 yers durtion presented with progressive pressure symptoms, nocturnl choking nd dyspne on exertion for 5 months. Physicl exmintion demonstrted lrge simple multinodulr goiter. Imging reveled deep retrosternl goiter extending below the trchel bifurction with mrked trchel devition. Totl thyroidectomy ws crried out vi cervicl pproch nd medin sternotomy. Extubtion ws not possible, nd the ptient hd to be kept intubted. She then went into mysthenic crisis. Initil ventiltory support ws followed by E-Mil krger@krger.com Europen Thyroid Assocition Published by S. Krger AG, Bsel /14/ $39.50/0 Mohmed E. Ahmed Deprtment of Surgery, Fculty of Medicine University of Khrtoum Khrtoum (Sudn) E-Mil us.net

2 intrvenous immunoglobulin, steroids nd pyridostigmine. The ptient hd complete remission nd ws symptomtic 18 months lter. Histopthology showed T-cell-rich thymom in ddition to nodulr colloid goiter Europen Thyroid Assocition Published by S. Krger AG, Bsel Introduction Goiter is endemic in mny prts of the Sudn nd thyroidectomy is common elective surgicl procedure in Khrtoum [1]. The presence of retrosternl opcity on chest X-ry with cervicl swelling lwys points to retrosternl extension of the thyroid. Thyroidectomy vi cervicl incision is often possible, however in deepseted goiter below the trchel bifurction, medin sternotomy my be resorted to [2]. The coexistence of retrosternl goiter nd thymom is n interesting combintion tht hs not been reported before. We present cse in whom thymom ssocited with retrosternl goiter ws initilly missed nd ws dignosed only when the ptient went into mysthenic crisis from which she recovered fter tretment nd remined well 18 months postopertively. Fig. 1. Trchel nrrowing due to side-to-side compression nd deep retrosternl extension of goiter nd presence of thymom. Cse Report A 32-yer-old Sudnese femle presented with goiter of 10 yers durtion. She hd experienced pressure symptoms for the lst 5 months with nocturnl choking, shortness of breth on lying flt nd hd to use two pillows while sleeping. She lso hd ftigue towrds the end of the dy long with progressive dysphgi for solids. Upper gstrointestinl endoscopy ws norml. There ws some degree of ptosis tht ws detected retrospectively fter the finl dignosis ws mde. Generl exmintion ws unremrkble. Cervicl exmintion showed simple multinodulr goiter mesuring cm with trchel devition to the left nd dull percussion note over the upper sternum indicting retrosternl extension. Upper gstrointestinl endoscopy ws norml. Investigtions showed norml blood picture, Hb 13 g/dl, totl WBC 6,000 cells/mm 3, cretinine 1.0 mg/dl, N 139 mmol/l, K 4 mmol/l, thyroid hormones T 3 95 nmol/l (norml rnge: ), T 4 9 nmol/l (norml rnge: ), nd TSH 2.5 mu/l (norml rnge: ). Plin chest X-ry showed deep retrosternl mss compressing the trche nd with mrked left side devition ( fig. 1 ). CT scn showed homogeneous nterior medistinl mss with distinct outlines nd no clcifiction or contrst enhncement which could be due to deep retrosternl extension of goiter below the trchel bifurction or lymphom ( fig. 2 ). The ptient hd totl thyroidectomy vi both cervicl collr incision nd medin sternotomy. We strted by cervicl pproch; the superior pole of the thyroid ws ligted nd trnsected. A tril of cervicl delivery by finger sweeping in the line of clevge nd gentle pulling ws ttempted on the left lobe but ws bndoned becuse the lower end hd brod bse nd ws dherent to the Fig. 2. CT scn showing retrosternl mss extending below the trchel bifurction. surrounding tissues including mjor vessels nd could not be delivered intct. The thyroid mss ws found to be seprte from nother retrosternl mss ( fig. 3 ). Delyed recovery from nesthesi led to clinicl suspicion of mysthenic crisis which ws confirmed cliniclly by response to dministrtion of pyridostigmine. Furthermore, lbortory tests confirmed the presence of cetylcholine receptor ntibodies. A trcheostomy ws crried out week lter nd the ventiltor support continued for 10 dys. Administrtion of intrvenous immunoglobulin 2 g/kg body weight divided over 5 dys led to improvement of the mysthenic symptoms. The ptient ws wened from the trcheostomy fter 3 weeks. The finl histopthology reported the thyroid tissue s nodulr colloid goiter with medistinl extension. The other medistinl mss consisted of nests nd sheets of cells with drk nuclei nd scnty cytoplsm ( fig. 4 ). In smll focus the cells exhibited poorly formed rosettes but there were no Hssll s corpuscles. There were scttered mitoses. By immunohistochemistry lmost ll the cells were positive for the T-cell mrker CD 3 ( fig. 4 b) but were Mysthenic Crisis Mnifesting s Postopertive Respirtory Filure 207

3 Fig. 3. Coexistence of retrosternl goiter ( ) nd thymom ( b ). b Color version vilble online Color version vilble online b Fig. 4. Nests nd sheets of smll cells with drk nuclei nd scnty cytoplsm. There re few poorly formed rosettes. HE. 40. The cells re positive for the T-cell mrker CD3. Immunoperoxidse stin (IPS). 40. b Most of the cells re positive for the T-cell mrker CD3. IPS. 40. c. Cells re negtive for the B-cell mrker CD20. IPS. 40. c 208 Ahmed /Mhgoub /Alnedr /Mhdi / Alzubeir /El Hssn /Elmin /El Hssn

4 negtive for the B-cell mrker CD20 ( fig. 4 c), cytokertin 20 nd EMA. Despite the fct tht epithelil cells were not seen, the dignosis of T-cell-rich thymom ws mde. It is known tht epithelil cells my be scnty on this type of thymom. The dignosis of thymom ws confirmed when the ptient went into thymic crisis nd ws shown to hve cetylcholine receptor ntibodies. Therefter, the ptient received tretment for T-cell lymphom in six cycles of chemotherpy in the form of cyclophosphmide 1 g, vincristine 2 mg, drimycin 80 mg, prednisolone 40 mg nd llopurinol 300 mg. She hd been on regulr follow-up with the neurologist nd tking pyridostigmine 40 mg/dy. After 18 months the ptient ws still in remission from T-cell-rich thymom nd the ssocited mystheni grvis. Discussion The cervicl swelling with retrosternl extension is firly common presenttion of goiter. However, the pressure symptoms such s choking, shortness of breth nd dysphgi s result of the goiter msked the erly symptoms of mystheni grvis in the form of ftigbility towrds the end of the dy. Retrosternl, substernl, intrthorcic or medistinl re terms tht hve been used to describe goiter tht extends beyond the thorcic inlet. Retrosternl extensions hve been observed in 3 20% of the ptients undergoing thyroidectomy [3, 4]. In contrst, primry extrnodl sites of lymphoid neoplsms re rre nd ffect bout 5% of ptients with Hodgkin s lymphom. Extrnodl development is more common in non-hodgkin s lymphom nd my rech up to 33% [5]. Mystheni grvis nowdys includes heterogeneous utoimmune diseses with postsynptic defect of neuromusculr trnsmission s the common feture [6] nd very rrely is ssocited with lymphoblstic mlignncies. Thymic epithelil tumors cn be chllenging to mnge becuse of locl invsion of medistinl structures nd high recurrence rte [7]. Non-cutneous T-cell lymphoms re rre ggressive tumors. Peripherl T-cell lymphom, derived from post-thymic T cells, ccounts for 10 15% of non-hodgkin lymphoms [8]. By definition, ll ptients with mysthenic crisis re in respirtory filure due to muscle wekness nd require ventiltory ssistnce. A preopertive prediction score of postopertive mysthenic crisis following thymectomy ws introduced nd strtifiction of the ptient risk ws grded into four groups with grde 4 crrying 50% risk of developing mysthenic crisis. Our ptient in retrospect could be stged in group 1 with 6% risk of postopertive respirtory filure bsed on Ossermn stge IIA (mild mystheni grvis), BMI <28 nd durtion of symptoms <1 yer [9]. In nother study the risk prediction of postopertive mysthenic crisis ws bsed on the presence of bulbr plsy nd n extended surgery, nd both were present in our ptient [10]. The differentil dignosis of nterior medistinl msses includes thymom, tertom, thyroid disese, nd lymphom [8, 11]. Retrosternl goiter with pressure symptoms needs surgicl tretment [12 14]. Assessment of the medistinl mss will need both CT-guided fineneedle spirtion cytology. However, in our cse the presence of lrge goiter in ptient coming from n endemic region mde the preopertive dignosis of retrosternl goiter rther obvious. This ptient hd thymom in the posterior medistinum. Thymoms my ffect both the nterior nd posterior medistinum. During the surgicl procedure, intropertive ssessment of the mss indicted the need for medin sternotomy tht ws crried out nd the mss mcroscopiclly ppered different nd totlly seprte from the thyroid glnd. A review of the literture reveled totl of 25 cses reported s lymphom long with mystheni grvis nd only 4 of these were reported s being T-cell lymphom [13]. The dignosis of mysthenic crisis should be suspected cliniclly nd ptients with impending crisis must be dmitted to n intensive cre unit for respirtory support. The condition is fully reversible nd crries no long-term disbility if treted quickly nd ppropritely [15, 16]. The ssocition of retrosternl goiter with thymom is rre. Bker et l. [17] reported cse of intrthymic primry intrthorcic goiter in ptient with brest cncer which ws removed vi medin sternotomy. Finlly, while tretment of lymphoid mlignncies usully induces improvement or remission of the mystheni grvis symptoms, mystheni grvis occsionlly emerges s result of tretment [13]. Conclusion The coexistence of lrge goiter with intrthorcic swelling does not lwys men retrosternl goiter. Creful history-tking nd preopertive ssessment is mndtory to exclude other potentilly mlignnt conditions. Cution is needed to void fctors tht cn trigger mysthenic crisis. Disclosure Sttement The uthors hve no conflicts of interest to disclose. Mysthenic Crisis Mnifesting s Postopertive Respirtory Filure 209

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