Metachronous Eosinophilic Granuloma of Rib in an Adult Patient

Abstract

Eosinophilic granuloma (EG) is the unifocal osseous form of Langerhans cell histiocytosis (LCH), which usually affects the skull and long bones. Although it most commonly affects the pediatric age group, it can rarely be seen in adults. Skeletal involvement is common in adult patients, but isolated rib involvement is extremely rare. Differential diagnosis includes other osteolytic lesions such as Ewing’s sarcoma, tuberculosis, multiple myeloma, lymphoma, and primary bone malignancy. The diagnosis must be confirmed histopathologically. In addition to pathological Langerhans cells, inflammatory cells such as lymphocytes, eosinophils, and macrophages are observed in microscopy. Immunohistochemically, CD1a, S-100, and Langerin positivity are observed in biopsy and/or surgical excision material. Treatment options may vary depending on the localization and extent of the disease. In unifocal EG, close observation of the patient may be preferred, as well as surgical excision, radiotherapy, and intra-lesional steroid administration. The prognosis in patients with a single bone lesion is quite good compared to other groups. In this case report, we present a metachronous EG of rib developed in two different ribs by an interval of seven years, which were both surgically treated. In this mild variant of LCH, surgical resection with clean margins has a favorable outcome without the need for additional adjuvant therapy. Metachronous tumors may develop in isolated unifocal bone EGs, and long-term follow-up is mandatory.

Isolated Tuberculosis of the Ribs in an Immunocompetent Adult

“…Other possible conditions are TB (second most common), primary and secondary bone tumours, multiple myeloma, lymphoma, granulomatous diseases such as sarcoidosis, eosinophilic granulomas and infections with fungi or amoeba. [14][15][16][17] In our patient, the contact history, socioeconomic status, constitutional symptoms, lytic rib lesions, presence of cold abscess, a positive tuberculin skin test, very high erythrocyte-sedimentation rate and positive PCR favoured TB. X-rays are notorious in being normal for the early stages of a disease, 18 as in our patient, who had been evaluated earlier by three providers.…”

Multifocal eosinophilic granuloma with femoral epiphyseal lesion mimicking an aneurysmal bone cyst

“…Eosinophilic granuloma is a type of benign bone tumor, and the mildest subtype of Langerhans cell histiocytosis. This condition has been described in many different anatomic locations within the human body [2 , 3 , [6] , [7] , [8] , 11 , 12] , and may present either in a single or multiple foci. This disease typically affects the axial skeleton, however, may also affect long bones with the majority of lesions occurring in the diaphysis [1] .…”

“…It involves the axial skeleton and diaphysis long bones as well as the soft tissues adjacent to the bone causing localized bone pain and tender soft tissue swelling [4] . It has a slight male predominance most commonly affecting the children and young adults; however, it can rarely be seen in adults [5] , [6] , [7] .…”

“…Therefore, the diagnosis must be confirmed histopathologically and immunohistochemically with a biopsy. The sample obtained is characterized by the presence of clonal proliferation of myeloid dendritic cells with Langerhans cell morphology with surrounding small lymphocytes, eosinophils, and histiocytes [7 , 9] .…”

“…Treatment options may vary depending on the localization and extent of the disease. In unifocal eosinophilic granuloma, close observation of the patient as well as surgical excision, radiotherapy, intralesional administration of corticosteroid and chemotherapeutic agents like vinblastine and etoposide might be considered [7 , 10 , 11] . In some cases, spontaneous regression of the lesion has been reported [11] .…”