Abstract: No doubt it is rightly said that abdomen is a temple of surprises. The case presented here is one of the uncommon entity which a surgeon encounters . Abdominal  mass always presents a diagnostic dilemma to the treating surgeon because of various structures from which it can arise. Mesentric mass in particular is one such aspect which always poses a difficullty to surgeon because of the extension of the mesentry right across the abdomen from right iliac fossa to left hypochondrium. The mass of the mesentry ranges from cystic mass to solid mass. The case that follows is a solid mass which is an uncommon variety of lymph node neoplasm- Castleman disease.

Keywords : castleman disease, lymph node neoplasm

Case report:

A 45 year old female patient presented to the emergency department with lower abdominal pain for 20 days duration. She had no other symptoms corresponding to gastrointestinal system involvement. She had no comorbid illness and have undergone sterilization at 27years of age. She attained menopause 3 years ago and had no significant post menopausal symptoms. On examination she was moderatly built and had no pallor and jaundice. She was in no distress. On examination, abdomen was soft, no mass palpable, normal bowel sounds and no organomegaly. Per rectal examination was normal. Her cardiac examination and respiratory examination was normal and her vitals were within normal limits. Patient was admitted for observation.

On blood investigation, patient was found to be anemic Hb% – 8.6g%. Ultra sonogram of the abdomen revealed a well defined solid homogenous mass 5.9X4.8cm noted in right paraumbilical region moving with respiration. CECT (contrast enhanced computed tomography)of  abdomen showed a 6.7 X 5 cm hypervascular lesion in the mesentry in the right lumbar region closely abutting the inferior wall of proximal transverse colon – with the possibilities of lymph node or carcinoid and multiple homogenously enhancing discrete lymph node in the mesentry in peripancreatic , perigastric and near transverse colon with the possibility of metastases [Fig.1]. OGD (oesophago gastro duodeno) scopy was done which showed antral gastritis. CT (computed tomogram)guided FNAC (fine needle aspiration cytology) of the mass was inconclusive and only hemorrhagic fluid was present.

An exploratory laparotomy was planned. Under general anesthesia  via midline incision, abdomen was explored and a firm mass of size 5X6 cm was present in the transverse mesocolon abutting the inferior surface of the proximal part of transverse colon [Fig.2]. Rest of the abdomen was normal. Mass was excised and sent for HPE(histopathological examination).  Abdomen was closed and post operative period was uneventful and patient was discharged on 10th post operative day. The biopsy report showed a solid mass with homogenous surface [Fig.3]and microscopic examination revealed a hyperplasia of lymphoid follicles with lollypop appearance and onion skin appearance[Fig. 4]increased hyaline collagen and a diagnosis of Castleman disease of hyaline variety made.

Discussion:

Castleman disease is a lymphoproliferative disorder first coined by Benjamin Castleman in 1956. It was formerly called as angiofollicular hyperplasia. It can be classified as unicentric vs mulitcentric ,  hyaline vs plasmacytic vs mixed cellularity and HIV negative vs positive[1]. The patient above discussed was unicentric, HIV negative and hyaline type. Unicentric tumors are usually located in mediastium or mesentry and symptoms are only due to pressure effects due to the bulkiness of the tumor. Multicentric variety on the other hand is widespread and presents with constitutional symptoms of fever , weight loss, fatigue and night sweats  and peripheral edema. Untreated multicentric variety progress to non hodgkins lymphoma.  Patients with multicentric disease usually have plasmacytic and mixed histology . If it is complicated by HIV the disease runs a very aggressive course. Human herpes virus 8(HHV 8) [2] is considered to be one of the causative agent of multicentric castleman disease because of its presence in lymph node and mononuclear cells in HIV positive patients. Detection of HHV 8 in HIV positive DNA antedates the development of multicentric disease by 5% to 35%. Cases of extrathoracic Castlemans disease with paraneoplastic phempigus and myasthenia gravis have been documented[3]. Curative therapy for unicentric disease is mainly surgical excision[4],[Fig.5- Post op status] and usually there is no progression to other tumors like lymphoma. If the lesion is too bulky pre operative steroids and rituxan can be tried. Radiotherapy also forms an important modiality of treatment in patients with unicnentric disease where surgery is not possible due to fixity to underlying structures and the response rate is near to 70%[5,6]. In case of multicentric disease it is mainly systemic therapy with intravenous immunoglobulins against Interlukin 6 ,Siltuximab [7,8]and other like rituximab for TAFRO (thrombocytopenia, anasarca, fever , reticulin fibrosis and organomegaly) [9]  . In intractable cases stem cell transplantation can be tried.

 

 

Conclusion:

The Patient is under regular followup for past 4yrs and is disease free . Latest CT abdomen showed normal study except few subcentimetric nodes in mesentry and in retroperitoneum. Though the presentation was clinically insignificant further investigations turned out to show one of the rare causes of lymphoproliferative disorder. Diagnosis of this disease is based on histopathological examination. The prognosis of  unicentric castleman disease is very good, close to 100% disease free survival rate  by 5years. On the other hand multicentric disease runs an aggressive course and at a risk of developing multiple autoimmune diseases like autoimmune hemolytic anemia, pemphigus ,amyloidosis and multiple myeloma. Thus this patient requires a life long follow up to find out possiblilty of unicentric transforming into a multicentric variety in a HIV negative patient.

 

 

 

Acknowledgement:

Prof.Dr.N.Ckaravarthy ., M.S(Gen Surg)

HOD of General Surgery,

Saveetha Medical College and Hospital,

Saveetha Nagar,

Thandalam,

Chennai.


 

References:

1.Multicentric Castlemans Disease and Kaposi sarcoma in a HIV positive patient on highly active retroviral therapy.

Ortega L, Cooper CJ, Otoukesh S, Mojtahedzadeh M, Didia CS, Torabi A, Nahleh Z.

Rare Tumors. 2014 Aug 6;6(3):5480. doi: 10.4081/rt.2014.5480. eCollection 2014 Jul 30. PMID: 25276327

 

 

2.Lymphoid Hyperplasia and Lymphoma in KSHV K1 transgenic mice

Berkova Z, Wang S, Sehgal L, Patel KP, Prakash O, Samaniego F.

Histol Histopathol. 2014 Oct 10. [Epub ahead of print] PMID:25301266

 

3. Castlemans disease with paraneoplastic phempigus .

Dinesha, Padyana M, Nayak K, Nirupama M, Pai DS.

Indian J Dermatol. 2014 Jul;59(4):421. doi: 10.4103/0019-5154.135528.

PMID:25071278[ Pubmed]

 

4. The management of unicentric and multicentricCastleman’s disease: a report of 16 cases and a review of the literature.

Bowne WB, Lewis JJ, Filippa DA, Niesvizky R, Brooks AD, Burt ME, Brennan MF.Cancer. 1999 Feb 1; 85(3):706-17.

 

5.The Role of Radiotherapy Among The Therapeutic Options for Castlemans Disease .

Karaca F, Usul Afşar C, Erkurt E, Arslantaş HS, Calış E, Totan Ateş B, Bağır E, Ergin M, Paydaş S.

Turk J Haematol. 2014 Jun;31(2):197-8. doi: 10.4274/tjh.2013.016. Epub 2014 Jun 10. No abstract available. PMID: 25035682

 

6. Cases report of unicentric Castleman’s disease: revisit of radiotherapy role.

Noh OK, Lee SW, Lee JW, Kim SY, Kim CS, Choi EK, Kim JH, Ahn SD.Radiat Oncol J. 2013 Mar; 31(1):48-54. Epub 2013 Mar 31.

 

7.Siltuximab for multicentric Castleman disease : a randomised , double blind, placebo controlled trial.

van Rhee F, Wong RS, Munshi N, Rossi JF, Ke XY, Fosså A, Simpson D, Capra M, Liu T, Hsieh RK, Goh YT, Zhu J, Cho SG, Ren H, Cavet J, Bandekar R, Rothman M, Puchalski TA, Reddy M, van de Velde H, Vermeulen J, Casper C.

Lancet Oncol. 2014 Aug;15(9):966-74. doi: 10.1016/S1470-2045(14)70319-5. Epub 2014 Jul 17. PMID:25042199 [Pubmed in Process].

 

8. Exploiting interlukin 6 in multicentric castlemans disease.

Bower M, Stebbing J.

Lancet Oncol. 2014 Aug;15(9):910-2. doi: 10.1016/S1470-2045(14)70333-X. Epub 2014 Jul 17. No abstract available. PMID : 25042200 [pubmed in process]

 

9. Efficacy of rituximab for TAFRO syndrome, a variant of multicentric castlemans disease.

Ozawa T, Kosugi S, Kito M, Onishi M, Kida T, Nakata S, Take H, Katagiri S.

Rinsho Ketsueki. 2014 Mar;55(3):350-5. PMD:2468190

 

 

Authors:

1 Godwin Devadhas Packiaraj.,M.S(Gen Surg) , FMAS

Assistant Professor , Department of General Surgery

Saveetha Medical College and Hospital

Saveetha nagar

Thandalam

Chennai – 602105

Ph.no. 9841204436

drgodwin80@gmail.com

 

2 P B Sudarshan.,M.S(Gen Surg),FIAGES

Associate Professor , Department of General Surgery

Saveetha Medical College and Hospital

Chennai – 602105

pbsudarshan@yahoo.co.in

 

 

3 Sridevi Manian.,M.D(Path)

Assistant Professor , Department of Pathology

Saveetha Medical College and Hospital

Chennai – 602105

dr.sridevi81@yahoo.com

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