Coexistence of Sarcoidosis and Malignancy

LTC (ret) William E. Caras, MD, FCCP, COL (ret) Thomas Dillard, MD, FCCP, LTC Thomas Baker, MD, COL (ret) Jerry Pluss, MD, FCCP


South Med J. 2003;96(9) 

In This Article

Case Reports

A 32-year-old black man was evaluated in the pulmonary clinic for an abnormal chest film. The film showed bilateral hilar and right paratracheal adenopathy consistent with Stage 1 sarcoidosis and was unchanged from a chest x-ray (CXR) obtained 3 years previously in 1990 (Fig. 1). Laboratory evaluation was remarkable for an elevation of serum angiotensin-converting enzyme level of 64 U (normal range, 8-52 U/L). A transbronchial biopsy revealed mononuclear inflammation and focal fibrosis but no clearly defined granuloma. He remained asymptomatic until 1996, at which time he noted fatigue, night sweats, and unintentional weight loss. A chest film showed a markedly enlarged right paratracheal node complex with bilateral widening of the superior mediastinum (Fig. 2). The hilar adenopathy remained unchanged. A punch biopsy of a skin rash along the dorsum of the neck revealed multiple well-defined granulomas (Fig. 3). He was given a 2-week course of corticosteroids but showed no interval resolution of his mediastinal adenopathy. A cervical mediastinal exploration with lymph node excision was obtained revealing Hodgkin's disease (HD), nodular sclerosing subtype (Fig. 4). He received six courses of alternating ABVD/MOPP combination chemotherapy-course 1, Adriamycin (57 mg), bleomycin (23 U), Velban (14 mg), and DTIC (848 mg); course 2, Mustargen (13.6 mg), vincristine (2.0 mg), procarbazine (225 mg), and prednisone (90 mg)-in addition to mantle radiation to the mediastinum (4,140 rad). During a follow-up gallium scan, uptake was noted in both mediastinal nodes and the left scapula. Because there was no other evidence of recurrent HD, he underwent an excisional bone biopsy. The bone biopsy specimen contained marrow elements and multiple noncaseating granulomas without evidence of HD (not shown). Four years later, he remained in clinical remission with stable anterior and subcarinal mediastinal adenopathy by chest computed tomographic (CT) scanning.

Chest film (posteroanterior and lateral) of Patient 1 showing hilar and right paratracheal enlargement.

Chest film (posteroanterior and lateral) of Patient 1 obtained 3 years after initial presentation. Note marked widening of the superior mediastinum and interval enlargement of the right paratracheal node complex.

Skin biopsy specimen from Patient 1 showing multiple noncaseating granulomas (hematoxylin and eosin; original magnification, x40).

Excisional mediastinal lymph node biopsy specimen from Patient 1 showing Hodgkin's disease (hematoxylin and eosin; original magnification, x40). Inset, Reed-Sternberg cell (hematoxylin and eosin; original magnification, x100).

Both sarcoid and HD are documented histologically. Noncaseating granulomas are occasionally found in HD and other malignancies as a nonspecific reaction (see Discussion section). In this case, however, bilateral hilar adenopathy preceded the diagnosis of HD by 3 years. After treatment and remission of HD, mediastinal and hilar adenopathy have remained stable for over 4 years. This radiographic history along with the presence of multiple noncaseating granulomas at two distinct tissue sites is most consistent with sarcoidosis.

A 38-year-old black man presented to the pulmonary clinic with a 9-year history of exertional dyspnea, which began in 1991 shortly after he returned from assignment in Saudi Arabia. For the same period, he had noted a sporadic cough that on occasion was productive of colored phlegm. In the past 4 months, he has noted progressive abdominal distention without associated pain, changes in bowel habit, or jaundice. Physical examination disclosed the presence of ascites without organomegaly. CXR and chest CT scan were remarkable for hilar and paratracheal adenopathy along with perihilar consolidations (not shown). The patient underwent bronchoscopy and paracentesis. Bronchoalveolar lavage disclosed many macrophages and rare multinucleated giant cells but no cytologic evidence of malignancy. Transbronchial biopsies showed multiple ill-defined noncaseating granulomas consistent with pulmonary sarcoidosis (Fig. 5). Special stains and cultures were negative for acid-fast and fungal organisms. The peritoneal fluid contained malignant cells consistent with an adenocarcinoma (Fig. 6). One week later, an endoscopy biopsy specimen from the gastric fundus was positive for moderately differentiated adenocarcinoma with focal signet ring features (not shown). Due to the advanced nature of his malignancy, the patient was treated with supportive nutritional care (percutaneous endoscopic gastrostomy tube) and died 4 months later in a hospice center. Permission for autopsy was not given.

Transbronchial biopsy specimen from Patient 2 showing scattered noncaseating granulomatous inflammation (hematoxylin and eosin; original magnification, x10).

Peritoneal fluid from Patient 2 showing malignant cells consistent with an adenocarcinoma (carcinoembryonic antigen immunoperoxidase; original magnification, x100).

Again, both sarcoid and malignancy are documented histologically. The 9-year history of cough and dyspnea along with the CT findings are consistent with Stage 2 pulmonary sarcoidosis. Bronchoalveolar lavage and transbronchial biopsy both supported a diagnosis of sarcoid and showed no evidence of carcinoma. The coexistence of sarcoid and gastric carcinoma is rare. Some authors have speculated that chronic sarcoid of the gastric mucosal lining may predispose to gastric malignancy.[2]

A 56-year-old man originally presented in July 2000 with right-sided "pulling" flank pain. He underwent abdominal and pelvic CT scanning that disclosed a large right retroperitoneal mass with adjacent spread to the right renal hilum. A chest film disclosed a right-sided pleural effusion and prominent hila bilaterally. An excisional lymph node biopsy from the left inguinal region showed non-Hodgkin's lymphoma (NHL) of the large cell subtype (Fig. 7A). After a negative bone marrow biopsy and aspiration, he underwent eight cycles of CHOP chemotherapy (Cytoxan [1,650 mg], vincristine [2 mg] Adriamycin [110 mg], and prednisone [100 mg]) from August until December. Afterward, a surveillance positron emission tomographic scan disclosed uptake in the mediastinum. A chest CT scan obtained in February 2000 disclosed right hilar and subcarinal adenopathy (Fig. 8). A bronchoscopically directed transbronchial needle (Wang) aspiration revealed a mixed population of lymphoid cells without clear evidence of malignancy. Central mediastinal exploration with lymph node excision disclosed scattered noncaseating granulomas without evidence of malignancy (Fig. 7B). Special stains were negative for acid-fast and fungal organisms. At his most recent follow-up appointment, he remained in remission nearly 1 year after his last treatment.

 A, inguinal lymph node biopsy specimen from Patient 3 showing NHL, large cell subtype (hematoxylin and eosin; original magnification, x40). B, excisional mediastinal lymph node biopsy specimen from the same patient showing noncaseating granulomata (hematoxylin and eosin; original magnification, x40).

CT scan of Patient 3 after the completion of cancer chemotherapy for NHL showing right hilar and subcarinal adenopathy.

In clinical practice, radiographic abnormalities in cancer patients are often presumed to represent metastasis. However, in this case, lymph node excision clearly showed noncaseating granulomas consistent with mediastinal sarcoid during a time of clinical remission from NHL. Retrospectively, the prominent hila noted on CXR at presentation may have represented coexistent sarcoidosis.


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