Hodgkin lymphoma remains primarily a nodal disease. Extranodal involvement in Hodgkin lymphoma is less common than that seen in non-Hodgkin lymphoma. In particular, extranodal Hodgkin lymphoma involving soft tissues is extremely rare. We report a case of extranodal Hodgkin lymphoma involving breast and thigh tissues in a 72 year-old female. CAT scan showed a complex mass like area centered around the distal aspect of the vastus medialis. Ultrasound showed an ovoid solid and cystic mass in the right breast. This case illustrates that, while rare, Hodgkin lymphoma can manifest as soft tissue masses

Deng, Aileen

Javad, Asad

Kasner, Margaret

Uppal, Guldeep

University of Missouri: MOspace

5/30/2018 An unusual case of late recurrent Hodgkin lymphoma presenting with soft tissue masses - American Journal of Hospital Medicinehttps://medicine2.missouri.edu/jahm/an-unusual-case-of-late-recurrent-hodgkin-lymphoma-presenting-with-soft-tissue-masses/ 1/7American Journal of Hospital MedicinePromoting research and education in the field of hospital medicine. ISSN 2474-7017 (online)Case ReportsAn unusual case of late recurrent Hodgkin lymphoma presenting withsoft tissue massesJune 30, 2015 Case Reports, Issues, July-September 2015: Volume 7 Issue 3Keywords  hodgkin lymphomaAileen Deng M.D., Asad Javad  M.D., Guldeep Uppal M.D., Margaret Kasner M.D.An unusual case of Hodgkin lymphoma presenting with soft tissue massesCase reportDepartment of Internal MedicineThomas Jefferson University HospitalCorresponding author:Aileen Deng M.D.833 Chestnut Street Suite 701Philadelphia, PA 19107732-547-0531Aileen.deng@jefferson.eduHodgkin lymphoma remains primarily a nodal disease. Extranodal involvement in Hodgkin lymphoma is lesscommon than that seen in non-Hodgkin lymphoma. In particular, extranodal Hodgkin lymphoma involving softtissues is extremely rare. We report a case of extranodal Hodgkin lymphoma involving breast and thigh tissues ina 72 year-old female. CAT scan showed a complex mass like area centered around the distal aspect of the vastusmedialis. Ultrasound showed an ovoid solid and cystic mass in the right breast. This case illustrates that, whilerare, Hodgkin lymphoma can manifest as soft tissue masses.An Unusual Presentation of Late Relapse Hodgkin Lymphoma with Thigh and Breast MassesIntroductionHodgkin lymphoma is a potentially curable lymphoma with a reported five-year event-free survival rangingbetween 80% and 90% with combined chemotherapy and radiotherapy[1]. However, 10% to 15% of patientswith localized and 25% to 30% with disseminated classical Hodgkin lymphoma fail to respond or relapse afterprimary conventional treatment[1,2]. Late relapse of Hodgkin’s lymphoma more than 5 years after completeremission following radiation therapy occurs in 5% to 10% of patients, and is more commonly seen followingcombined modality therapy[3]. While extra nodal involvement of Hodgkin lymphoma can occur in up to 30% ofcases, skin and musculoskeletal involvement are extremely rare[4]. We report a case report of late relapse ofHodgkin lymphoma presenting with breast and thigh mass.Case Presentation5/30/2018 An unusual case of late recurrent Hodgkin lymphoma presenting with soft tissue masses - American Journal of Hospital Medicinehttps://medicine2.missouri.edu/jahm/an-unusual-case-of-late-recurrent-hodgkin-lymphoma-presenting-with-soft-tissue-masses/ 2/7A 72 year-old Caucasian female presented with a progressively enlarging left thigh mass associated withlocalized pain and fevers. Approximately 3 months prior to presentation, the patient initially noted a skin noduleon her left anterior thigh. The patient reported that, since then, the overlying skin nodule had gradually enlargedinto a palpable, painful mass.The patient has a history of stage IIA nodular sclerosing Hodgkin lymphoma that presented with mediastinaladenopathy and was diagnosed ten years prior to her current presentation. It was treated with doxorubicin,bleomycin, vinblastine, and dacarbazine (ABVD) chemotherapy regimen and involved-field radiotherapy. Inaddition, she has a history of diffuse large B-cell non-Hodgkin lymphoma, lymphomatoid granulomatosis thatwas diagnosed 5 years prior to presentation and treated with rituximab plus cyclophosphamide, doxorubicin,vincristine, and prednisone (RCHOP) chemotherapy regimen.On presentation she had a soft, palpable left inguinal lymph node and a firm left thigh mass with overlying skinchanges. The overlying skin had a large area of central necrosis and erosion and a surrounding erythematous,maculopapular rash. In addition, she had a firm mass in the right breast with no overlying skin changes.Lab work included a white blood cell count of 7.5 B/L, hemoglobin of 11.4 g/dL, hematocrit of 34.1% andplatelet count of 142 B/L.CT of the left knee showed a complex mass like area centered around the distal aspect of the vastus medialiswith no definite involvement of the underlying femur (Figure 1). Patient underwent image-guided core biopsy ofthe soft tissue mass.Surgical pathology of left thigh mass revealed recurrent classical Hodgkin lymphoma (Figure 2A-2B). Onmicroscopy, the section revealed cores of soft tissue with atypical nodular lymphocytic infiltrate. Scattered largeatypical cells were present within the infiltrate. The atypical large cells were positive for CD30. The backgroundlymphocytes were predominantly T cells, positive for CD3.Ultrasound of breast mass showed ovoid solid and cystic mass measuring 2.4 x 0.9 x 1.9 centimeters at the 12:00position 6 cm from the nipple (Figure 3). Subsequently, patient underwent ultrasound-guided core biopsy of theright breast mass.Pathology of the right breast mass revealed recurrent classical Hodgkin lymphoma (Figure 2C-2D). Onmicroscopy, core biopsy revealed breast parenchyma with atypical lymphoid infiltrate. The infiltrate showed asmall number of atypical large cells admixed with lymphocytes and histiocytes. The atypical large cells werepositive for CD30. The background lymphocytes were T cells that were positive for CD3.CT scan of chest, abdomen and pelvis were completed for staging. It revealed new and increased nodules in theleft lung, a soft tissue density mass along the superior aspect of the right breast, splenomegaly with no focalsplenic lesions, left groin adenopathy and matted soft tissue mass in the left groin with associated subcutaneousedema extending the left lower extremity.Patient was started on ICE (ifofamide, carboplatin, and etoposide) regimen. She completed three cycles ICE,followed by maintenance brentuximab. Unfortunately, she has since developed progressive disease.5/30/2018 An unusual case of late recurrent Hodgkin lymphoma presenting with soft tissue masses - American Journal of Hospital Medicinehttps://medicine2.missouri.edu/jahm/an-unusual-case-of-late-recurrent-hodgkin-lymphoma-presenting-with-soft-tissue-masses/ 3/7Figure 1: CT scan of leftknee with nodular mass-like area in the medialleft thigh centered at thedistal aspect of thevastus medialisFigure 2A: Core biopsyof the thigh mass,vaguely nodular atypicallymphoid infiltrate (H &E stain, 200x originalmagnification) Figure 2B: Core biopsyof the thigh mass,scattered mononuclearHodgkin cells (greenarrows) and Mummifiedcells (black arrows) in amixed inflammatorybackground (H & Estain, 4000x originalmagnification) Figure 2C: Core biopsyof the breast lesion,showing identicalmorphology to thighbiopsy with scatteredHodgkin cells against apolymorphicbackground (H & Estain, 4000x originalmagnification) Figure 2D: Core biopsyof the breast lesion,scattered Hodgkin cellswith membrane andGolgi zone staining withCD30 antibody (CD30immunostain, 4000xoriginal magnification)5/30/2018 An unusual case of late recurrent Hodgkin lymphoma presenting with soft tissue masses - American Journal of Hospital Medicinehttps://medicine2.missouri.edu/jahm/an-unusual-case-of-late-recurrent-hodgkin-lymphoma-presenting-with-soft-tissue-masses/ 4/7Figure 3: Ultrasound of right breast with an ovoid solid and cystic massDiscussionClassical Hodgkin’s lymphoma tends to cause mass lesions, most commonly in the centriaxial lymph nodes, butoccasionally in other organs[5]. At the time of diagnosis, the majority of patients with Hodgkin lymphomapresent with supra-diaphragmatic lymphadenopathy. Approximately one-third of patients presents with systemicsymptoms including fever, night sweats, weight loss or chronic pruritus[6].Extranodal presentation of Hodgkin lymphoma with or without lymphatic involvement occurs in 15% to 30% ofcases[7]. Hodgkin lymphoma affects extranodal tissues by direct invasion or by hematogenous spread[6]. Themost commonly involved extranodal sites include the spleen, lung, liver and bone marrow. Rare cases at otherextranodal sites such as skin, brain, gastrointestinal tract, or musculoskeletal tissue constitute less than 1% ofHodgkin lymphoma presentation[4].Extranodal Hodgkin lymphoma presenting with soft tissue masses is rare. In the review of the literature, onlytwo case reports have been written on Hodgkin lymphoma presenting with soft tissue mass in the extremity. A33 year-old male presented with numerous soft tissue masses in the subcutaneous tissue of the left arm[8]. MRIimaging revealed multiple soft tissue masses with the largest mass measuring 25 x 35 x 58 mm localized alongthe neurovascular bundle and the ipsilateral axillary region. Excisional biopsy of a soft tissue mass led to adiagnosis of lymphocyte-rich Hodgkin lymphoma. Another case report described a patient who presented with bilobulated, subcutaneous mass on the medial aspect of the right arm with normal adjacent bone andneurovascular structures[9]. The patient was ultimately diagnosed with lymphocyte-predominant Hodgkinlymphoma.Extranodal Hodgkin lymphoma presenting with soft tissue masses have been described in other body regions. A38 year-old Chinese female who presented with pain at the right upper side of the chest and adjacent soft tissueswelling was found to have an osteolytic lesion in the right second rib with an associated large soft tissue massmeasuring 6 x 5 x 5 cm in size[10]. She was diagnosed with primary osseous Hodgkin lymphoma. A 68 year-oldmale presented with a gluteal mass and hypercalcemia[11]. MRI imaging revealed a 15 cm mass infiltrating thegluteal and iliopsoas muscles, which was diagnosed to be nodular sclerosing Hodgkin disease. A 53 year-oldMoroccan woman was found to have a mediastinal mass protruding through the sternum, which was laterdiagnosed as nodular sclerosing Hodgkin lymphoma[12].5/30/2018 An unusual case of late recurrent Hodgkin lymphoma presenting with soft tissue masses - American Journal of Hospital Medicinehttps://medicine2.missouri.edu/jahm/an-unusual-case-of-late-recurrent-hodgkin-lymphoma-presenting-with-soft-tissue-masses/ 5/7Hodgkin lymphoma presenting with soft tissue mass in the breast is rare[13]. Less than 0.5% of all malignantlymphomas have secondary breast involvement[14]. It is more common in metastatic disease versus primarylymphoma[15–17].  In a study looking at 106 patients with breast lymphomas, 1 of the 50 patients with localizedbreast lymphoma had classical Hodgkin lymphoma while 3 of the 56 patients with disseminated breastlymphoma had classical Hodgkin lymphoma[18]. Few cases report disseminated Hodgkin lymphoma presentingwith breast involvement. A 33 year-old patient presented with recurrent Hodgkin lymphoma with disseminatedlymphadenopathy, bilateral breast involvement and bone and bone marrow involvement[16]. Ultrasound of thebreasts showed multiple hypoechoic solid masses in the left breast and one hypoechoic mass in the right breast.A 25 year-old female with a history of nodular sclerosing Hodgkin lymphoma presented left cervical, axillary,and inguinal lymphadenopathy with left breast mass[19].Our patient is unique in her presentation and her age. In the review of the literature, our patient is a rarepresentation of recurrent Hodgkin lymphoma presenting with thigh and breast mass. Soft tissue masses are morecommonly reported with non-Hodgkin lymphoma. However, our case illustrates that Hodgkin lymphoma canalso present with soft tissue masses. While most cases of Hodgkin lymphoma presenting with soft tissue massesoccurred in younger patients, our case illustrates the possibility of such presentation in older patients. Althoughrare, in older patients who present with soft tissue masses, Hodgkin lymphoma should be amongst thedifferential diagnosis.References[1]         Ferme C. Intensive Salvage Therapy With High-Dose Chemotherapy for Patients With AdvancedHodgkin’s Disease in Relapse or Failure After Initial Chemotherapy: Results of the Groupe d’Etudes desLymphomes de l’Adulte H89 Trial. J Clin Oncol [Internet]. 2002 Jan 15 [cited 2015 May 26];20(2):467–75.Available from: http://jco.ascopubs.org/content/20/2/467.short[2]         Lohri A, Barnett M, Fairey RN, O’Reilly SE, Phillips GL, Reece D, et al. Outcome of treatment of firstrelapse of Hodgkin’s disease after primary chemotherapy: identification of risk factors from the BritishColumbia experience 1970 to 1988. Blood [Internet]. 1991 May 15 [cited 2015 May 26];77(10):2292–8.Available from: http://www.ncbi.nlm.nih.gov/pubmed/1709382[3]         Ilan Y, Ruchlemer R, Lugassy G, Uzielly B, Williams SF, Golomb HM, et al. Late Relapse in Hodgkin’sDisease: Report of Five Cases and a Review of the Literature. Informa UK Ltd  UK; 2009 Jul 1 [cited 2015 May26]; Available from: http://informahealthcare.com/doi/abs/10.3109/10428199109068134[4]         Swerdlow S, Campo E, Harris N, Pileri S. WHO Classification of tumours of haematopoietic andlymphoid tissues. Lyon, Fr IARC [Internet]. 2008 [cited 2015 Mar 3]; Available from:http://scholar.google.com/scholar_lookup?title=WHO Classification of Tumours of Haematopoietic andLymphoid Tissues&author=SH Swerdlow&author=E Campo&author=NL Harris&publication_year=2008#0[5]         Connors J. Clinical manifestations and natural history of Hodgkin’s lymphoma. Cancer J [Internet].2009 [cited 2015 May 26]; Available from:http://journals.lww.com/journalppo/Abstract/2009/04000/Clinical_Manifestations_and_Natural_History_of.6.aspx[6]         Ansell S. Hodgkin lymphoma: 2012 update on diagnosis, risk‐stratification, and management. 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J Nat  … [Internet]. 2014 [cited 2015 May 26]; Available from:http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4121938/Main MenuAboutCurrent IssuePast IssuesGoalsEditorial BoardInstructions for authorsSearchSearch for:  Search5/30/2018 An unusual case of late recurrent Hodgkin lymphoma presenting with soft tissue masses - American Journal of Hospital Medicinehttps://medicine2.missouri.edu/jahm/an-unusual-case-of-late-recurrent-hodgkin-lymphoma-presenting-with-soft-tissue-masses/ 7/7LinksAdvanced Hospital Medicine FellowshipPhysician Assistant Residency in Hospital MedicineCopyright © 2018 - The Curators of the University of Missouri System. All rights reserved. DMCA and other copyright information. An equal opportunity/affirmative action institution.

An unusual case of late recurrent Hodgkin lymphoma presenting with soft tissue masses

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An unusual case of late recurrent Hodgkin lymphoma presenting with soft tissue masses

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