GRAY SCALE ULTRASOUND OF LYMPHOMA
COMPLICATING
LYMPHOMATOID HIROTSUGU ARTHUR
JAMES
GRANULOMATOSIS
MUNECHIKA,
T. ROSENFIELD,
KENNETH
J. W. TAYLOR,
W. WALSH,
C. ELTON
CAHOW,
IN IDENTIFICATION
M.D. M.D. M.D.,
PH.D.
M.D. M.D.
From the Departments of Diagnostic Radiology Surgery, Yale University School of Medicine, New Haven, Connecticut
and
ABSTRACT - Lymphomatoid granulomatosis is a necrotizing vasculitis involving multiple organs which may be associated with lymphomatous transformation. A case of lymphomatoid granulomatosis is presented in which gray scale ultrasound identijed lymphomatous transformation involving the right kidney and retroperitoneum, thus preventing unnecessary surgery. Ultrasound is a valuable modality for identifying and characterizing abdominal masses in patients at risk for neoplastic disease.
Lymphomatoid granulomatosis is a recently described necrotizing vasculitis of unknown etiology which involves predominately the lung and may also involve the reticuloendothelial system, the central nervous system, the kidneys, or the adrenals. l-3 Lymphomatous transformation may occur in this entity. 3 We describe the role of gray scale ultrasound in identifying lymphoma complicating lymphomatoid granulomatosis. Case Report A twenty-seven-year-old woman with a three-week history of fever was hospitalized. Physical examination demonstrated small papules on the skin which were distributed over the back, upper trunk, and extremities. Her chest x-ray film demonstrated a diffuse reticulonodular infiltrate predominately in the lower lobes. Open lung biopsy, skin biopsy, and cervical lymph node biopsy all demonstrated findings typical of lymphomatoid granulomatosis.
UROLOGY / APRIL 1979 / VOLUME XIII, NUMBER4
The patient was admitted to the Yale-New Haven Hospital six months later with right abdominal pain and hematuria. Chest x-ray film at this time demonstrated interstitial pulmonary disease, predominately at the bases, and bilateral mediastinal and hilar lymphadenopathy (Fig. 1A). Excretory urogram showed a nonfunctioning right kidney (Fig. 1B). Gray scale ultrasonography was performed to evaluate further the right kidney and retroperitoneum. The ultrasound study revealed a marked enlargement of the lower pole of the right kidney (Fig. 2). The normal differentiation between cortex and medulla in this region could not be appreciated, and the differential diagnosis lay between renal vein thrombosis and tumor. A space-occupying mass was noted in the prevertebral region immediately anterior to the superior mesenteric vessels and caudal to the pancreas (Fig. 3A). The mass was homogenous and was considered to be consistent with a lymphomatosis or sarcomatosis tumor. The demonstration of this prevertebral
443
FIGURE 1. (A) Chest x-ray film taken at time of admission to the Yale-New Haven Hospital demonstrates basilar interstitial pulmonary disease and bilateral mediastinal and hilar adenopathy. (B) Excretory urography demonstrates nonfunctioning right kidney and minimal lateral deviation of upper left ureter.
FIGURE 2. Longitudinal parasagittal ultrasound section through liver (L) and right kidney. Inferior portion of right kidney enlarged (arrowheads) and demonstrates abnormal pattern with no distinction between cortex and medulla. Finding consistent with infiltrating lesion of renal parenchyma.
444
tumor suggested that the process in the right kidney was of similar nature. Over the next three days, the prevertebral mass became palpable in the epigastrium, and a repeat ultrasound was requested. This showed a marked increase in the size of the mass; this rapid increase suggested that the appearances were due to hemorrhage into a pre-existent tumor (Fig. 3B). The right renal mass appeared unchanged in size but did produce an indentation of the inferior vena cava (Fig. 4). Renal arteriography demonstrated an avascular mass in the lower pole of the right kidney and right renal vein thrombosis. The renal mass was considered to be secondary to this renal vein thrombosis. However, the ultrasound findings had indicated nodal involvement by tumor with hemorrhage into the tumor and probable tumor in the right kidney. These findings were consistent with lymphomatous transformation of the lymphomatoid granulomatosis. In view of this, surgery was not considered necessary. The patient was treated with chemotherapy but had a downhill course and died three weeks after admission. At autopsy, malignant lymphoma was found involving the inferior portion
UROLOGY
/ APRIL 1979 / VOLUME
XIII.
NUMBER 4
FIGURE 3. Longitudinal parasagittal sections through left upper quadrant (A) at level of aorta (A’). Solid mass (arrowheads) noted anterior to superior mesenteric artery (black arrow) and vein (white arrow) and inferior to liver (L). (B) F our days after ultrasound study (Fig. 2) shows mass (arrowheads) anterior to superior These findings suggest hemorrhage into mass mesenteric vessels has increased in size and is more sonolucent. has occurred (L = liver, A’ = aorta).
of the right kidney, and there was right renal vein thrombosis due to tumor involvement of the adjacent para-aortic lymph nodes. Involved nodes were also found in the mediastinum and retroperitoneum. The palpable mass in the epigastrium was shown to be lymphadenopathy affecting both the para-aortic and mesenteric nodes, which contained obvious areas of frank necrosis.
phomatous transformation is a known complication. If this occurs, the treatment generally must be altered. Gray scale ultrasound is ideally suited to screen for abdominal masses. Retroperitoneal masses which are relatively sonolucent are typical of either a lymphomatous or sarcomatous process. When they are seen in lymphomatoid granulomatosis, they should
Comment Lymphomatoid granulomatosis was originally classified as a limited form of Wegner granulomatosis but later was reclassified by Liebow as a specific disease.’ It is characterized pathologically by an angiocentric, angiodestructive, lymphoreticular, and proliferative lesion. The lung is the primary area of involvement. Interstitial lesions or well-circumscribed masses may be seen. The clinical course is variable and may be characterized by a rapid death or a long remission. Cough, fever, and dyspnea are typical symptoms of lung involvement. The kidneys, skin, central nervous system, reticuloendothelial system, and adrenal glands may be affected with a polymorphous infiltrate which generally consists of lymphocytes, lymphoblasts, and plasma cells. Biopsy is necessary to make the diagnosis. Few cases have been studied and, therefore, the natural history is not well defined. Lym-
UROLOGY
/ APRIL1979 / VOLUME
XIII,NUMBER4
FIGURE 4. Longitudinal parasagittal section through liver (L) at level of inferior vena cava shows cava bowed forward (arrow) by mass posterior to it.
445
permit the correct diagnosis of lymphoma complicating this entity. In our patient, the retroperitoneal process was identified by ultrasound before it was clinically suspected and allowed the correct diagnosis of hemorrhage into this pre-existent tumor when the mass became clinically palpable. Ultrasound is a rapid, noninvasive modality which does not involve ionizing radiation. Serial examinations can be done in patients with lymphomatoid granulomatosis in an attempt to identify a lymphomatosis process early in the course of this disease. Ultrasound is also capable of examining the abdominal organs such as spleen, liver, and kidneys to evaluate them for neoplastic involvement.
446
333 Cedar Street New Haven, Connecticut 06510 (DR. ROSENFIELD) ACKNOWLEDGMENTS: To Dr. Fred and Joan Dimow for editorial assistance.
Kantor
for advice,
References 1. Liebow AA, Carington CB, and Freidman PJ: Lympbomatoid granulomatosis, Human Pathol. 3: 457 (1972). 2. Liebow AA: The J. Bums Amberson lecture; pulmonary angiitis and granulomatosis, Am. Rev. Respir. Dis. 108: 1 (1973). 3. Lee SC, Roth LM, and Brashear RE: Lymphomatoid granulomatosis: a cliniocopathologic study of four cases, Cancer 38: 646 (1976). 4. Heitzman ER, Markarian B, and DeLise CT: Lymphoproliferative disorders of the thorax, Sem. Roentgenol. 10: 73 (1975).
UROLOGY
/ APRIL 1979
/
VOLUME
XIII,
NUMBER
4
COMPLICATING
LYMPHOMATOID HIROTSUGU ARTHUR
JAMES
GRANULOMATOSIS
MUNECHIKA,
T. ROSENFIELD,
KENNETH
J. W. TAYLOR,
W. WALSH,
C. ELTON
CAHOW,
IN IDENTIFICATION
M.D. M.D. M.D.,
PH.D.
M.D. M.D.
From the Departments of Diagnostic Radiology Surgery, Yale University School of Medicine, New Haven, Connecticut
and
ABSTRACT - Lymphomatoid granulomatosis is a necrotizing vasculitis involving multiple organs which may be associated with lymphomatous transformation. A case of lymphomatoid granulomatosis is presented in which gray scale ultrasound identijed lymphomatous transformation involving the right kidney and retroperitoneum, thus preventing unnecessary surgery. Ultrasound is a valuable modality for identifying and characterizing abdominal masses in patients at risk for neoplastic disease.
Lymphomatoid granulomatosis is a recently described necrotizing vasculitis of unknown etiology which involves predominately the lung and may also involve the reticuloendothelial system, the central nervous system, the kidneys, or the adrenals. l-3 Lymphomatous transformation may occur in this entity. 3 We describe the role of gray scale ultrasound in identifying lymphoma complicating lymphomatoid granulomatosis. Case Report A twenty-seven-year-old woman with a three-week history of fever was hospitalized. Physical examination demonstrated small papules on the skin which were distributed over the back, upper trunk, and extremities. Her chest x-ray film demonstrated a diffuse reticulonodular infiltrate predominately in the lower lobes. Open lung biopsy, skin biopsy, and cervical lymph node biopsy all demonstrated findings typical of lymphomatoid granulomatosis.
UROLOGY / APRIL 1979 / VOLUME XIII, NUMBER4
The patient was admitted to the Yale-New Haven Hospital six months later with right abdominal pain and hematuria. Chest x-ray film at this time demonstrated interstitial pulmonary disease, predominately at the bases, and bilateral mediastinal and hilar lymphadenopathy (Fig. 1A). Excretory urogram showed a nonfunctioning right kidney (Fig. 1B). Gray scale ultrasonography was performed to evaluate further the right kidney and retroperitoneum. The ultrasound study revealed a marked enlargement of the lower pole of the right kidney (Fig. 2). The normal differentiation between cortex and medulla in this region could not be appreciated, and the differential diagnosis lay between renal vein thrombosis and tumor. A space-occupying mass was noted in the prevertebral region immediately anterior to the superior mesenteric vessels and caudal to the pancreas (Fig. 3A). The mass was homogenous and was considered to be consistent with a lymphomatosis or sarcomatosis tumor. The demonstration of this prevertebral
443
FIGURE 1. (A) Chest x-ray film taken at time of admission to the Yale-New Haven Hospital demonstrates basilar interstitial pulmonary disease and bilateral mediastinal and hilar adenopathy. (B) Excretory urography demonstrates nonfunctioning right kidney and minimal lateral deviation of upper left ureter.
FIGURE 2. Longitudinal parasagittal ultrasound section through liver (L) and right kidney. Inferior portion of right kidney enlarged (arrowheads) and demonstrates abnormal pattern with no distinction between cortex and medulla. Finding consistent with infiltrating lesion of renal parenchyma.
444
tumor suggested that the process in the right kidney was of similar nature. Over the next three days, the prevertebral mass became palpable in the epigastrium, and a repeat ultrasound was requested. This showed a marked increase in the size of the mass; this rapid increase suggested that the appearances were due to hemorrhage into a pre-existent tumor (Fig. 3B). The right renal mass appeared unchanged in size but did produce an indentation of the inferior vena cava (Fig. 4). Renal arteriography demonstrated an avascular mass in the lower pole of the right kidney and right renal vein thrombosis. The renal mass was considered to be secondary to this renal vein thrombosis. However, the ultrasound findings had indicated nodal involvement by tumor with hemorrhage into the tumor and probable tumor in the right kidney. These findings were consistent with lymphomatous transformation of the lymphomatoid granulomatosis. In view of this, surgery was not considered necessary. The patient was treated with chemotherapy but had a downhill course and died three weeks after admission. At autopsy, malignant lymphoma was found involving the inferior portion
UROLOGY
/ APRIL 1979 / VOLUME
XIII.
NUMBER 4
FIGURE 3. Longitudinal parasagittal sections through left upper quadrant (A) at level of aorta (A’). Solid mass (arrowheads) noted anterior to superior mesenteric artery (black arrow) and vein (white arrow) and inferior to liver (L). (B) F our days after ultrasound study (Fig. 2) shows mass (arrowheads) anterior to superior These findings suggest hemorrhage into mass mesenteric vessels has increased in size and is more sonolucent. has occurred (L = liver, A’ = aorta).
of the right kidney, and there was right renal vein thrombosis due to tumor involvement of the adjacent para-aortic lymph nodes. Involved nodes were also found in the mediastinum and retroperitoneum. The palpable mass in the epigastrium was shown to be lymphadenopathy affecting both the para-aortic and mesenteric nodes, which contained obvious areas of frank necrosis.
phomatous transformation is a known complication. If this occurs, the treatment generally must be altered. Gray scale ultrasound is ideally suited to screen for abdominal masses. Retroperitoneal masses which are relatively sonolucent are typical of either a lymphomatous or sarcomatous process. When they are seen in lymphomatoid granulomatosis, they should
Comment Lymphomatoid granulomatosis was originally classified as a limited form of Wegner granulomatosis but later was reclassified by Liebow as a specific disease.’ It is characterized pathologically by an angiocentric, angiodestructive, lymphoreticular, and proliferative lesion. The lung is the primary area of involvement. Interstitial lesions or well-circumscribed masses may be seen. The clinical course is variable and may be characterized by a rapid death or a long remission. Cough, fever, and dyspnea are typical symptoms of lung involvement. The kidneys, skin, central nervous system, reticuloendothelial system, and adrenal glands may be affected with a polymorphous infiltrate which generally consists of lymphocytes, lymphoblasts, and plasma cells. Biopsy is necessary to make the diagnosis. Few cases have been studied and, therefore, the natural history is not well defined. Lym-
UROLOGY
/ APRIL1979 / VOLUME
XIII,NUMBER4
FIGURE 4. Longitudinal parasagittal section through liver (L) at level of inferior vena cava shows cava bowed forward (arrow) by mass posterior to it.
445
permit the correct diagnosis of lymphoma complicating this entity. In our patient, the retroperitoneal process was identified by ultrasound before it was clinically suspected and allowed the correct diagnosis of hemorrhage into this pre-existent tumor when the mass became clinically palpable. Ultrasound is a rapid, noninvasive modality which does not involve ionizing radiation. Serial examinations can be done in patients with lymphomatoid granulomatosis in an attempt to identify a lymphomatosis process early in the course of this disease. Ultrasound is also capable of examining the abdominal organs such as spleen, liver, and kidneys to evaluate them for neoplastic involvement.
446
333 Cedar Street New Haven, Connecticut 06510 (DR. ROSENFIELD) ACKNOWLEDGMENTS: To Dr. Fred and Joan Dimow for editorial assistance.
Kantor
for advice,
References 1. Liebow AA, Carington CB, and Freidman PJ: Lympbomatoid granulomatosis, Human Pathol. 3: 457 (1972). 2. Liebow AA: The J. Bums Amberson lecture; pulmonary angiitis and granulomatosis, Am. Rev. Respir. Dis. 108: 1 (1973). 3. Lee SC, Roth LM, and Brashear RE: Lymphomatoid granulomatosis: a cliniocopathologic study of four cases, Cancer 38: 646 (1976). 4. Heitzman ER, Markarian B, and DeLise CT: Lymphoproliferative disorders of the thorax, Sem. Roentgenol. 10: 73 (1975).
UROLOGY
/ APRIL 1979
/
VOLUME
XIII,
NUMBER
4
