Int J Hematol DOI 10.1007/s12185-015-1748-6
IMAGES IN HEMATOLOGY
Massive splenic hamartoma with bizarre stromal cells Nuri Yigit · Shannon Covey · Wayne Tam
Received: 28 November 2014 / Revised: 15 January 2015 / Accepted: 21 January 2015 © The Japanese Society of Hematology 2015
Keywords Splenic hamartoma · Bizarre stromal cells · Sickle cell trait
A 35-year-old female with history of sickle cell trait presented to the emergency room with chest pain and pelvic pressure. Blood test showed pancytopenia. She had already undergone a bone marrow biopsy, which was found with a norm cellular marrow with increased megakaryocytes. Computed tomography scan with contrast revealed massive splenomegaly, secondary to a heterogeneously hypo enhancing solitary lesion with multiple relatively hypo dense striations, measuring up to 24.5 × 13.6 × 13.0 cm. Differential included mainly hamartoma and lymphoma, with other benign and malignant entities considered less likely. The patient underwent splenectomy. Cut sections of spleen revealed a large well-circumscribed, dark red colored, soft and solid mass replacing nearly the entire spleen with a small rim of unremarkable parenchyma (Fig. 1a, b). Histopathological examination showed a
Present Address: N. Yigit (*) · S. Covey · W. Tam Department of Pathology and Laboratory Medicine, Weill Cornell Medical College/New York-Presbyterian Hospital, 525 East 68th Street Starr Pavilion, 715, New York, NY 10065, USA e-mail: [email protected]; [email protected] S. Covey e-mail: [email protected] W. Tam e-mail: [email protected] N. Yigit Department of Pathology, Gulhane Military Medical Academy and School of Medicine, Etlik Kecioren, Ankara 06010, Turkey
well-demarcated and encapsulated mass with focal infarcts, extensive hemorrhage, and extramedullary hematopoiesis, including erythropoiesis and megakaryopoiesis (Fig. 1c). Thick bands of hypocellular fibrosis, disorganized sinusoids, and vascular spaces separated by red-pulp stroma, without any white pulp elements, were observed. Many dispersed small lymphocytes, plasma cells, siderophage, and a few siderotic nodules (Gamna–Gandy bodies) were also noted in the background. The most striking population was scattered bizarre stromal cells, which were seen throughout the stroma without any association with vascular lumina. These cells composed of round to oval distorted or multilobulated nuclei, open chromatin, conspicuous nucleoli and scant cytoplasm (Fig. 1d). Large and pale intranuclear pseudo inclusions were frequently identified. Although their morphology was worrisome, they were devoid of some features of malignancy such as hyperchromasia, increased mitosis, or infiltrative growth pattern. An immunohistochemical panel containing CD34, CD31, CD8, CD163, CD138, Kappa, Lambda, CD30, S100, nerve growth factor receptor, smooth muscle actin, Desmin, and Pancytokeratin was performed. The bizarre cells were negative for all markers except focal and faint positivity for Desmin. Immunostains confirmed vascular and sinusoidal disorganization within the lesion. Plasma cells were polytypic for Kappa and Lambda light chains. Additionally, special stains for Grocott’s methenamine silver and acidfast bacilli were negative for any microorganism. Although these cells raised suspicion of malignancy, they were completely benign in nature. Their deceptively malignant morphology and lack of specific expression of any immunohistochemical marker are attributable to degenerative changes that can result from losing connections with microenvironmental elements and deteriorated nutritional supply [1].
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Fig. 1 a, b Large lesion contains extensive hemorrhage and infarct foci. The clear demarcation line is seen between lesion and small intact rim of splenic parenchyma (arrows). c The hamartoma are harboring an admixture of extensive hemorrhages, hemosiderin-
laden macrophages, erythropoiesis foci (upper left), and bizarre cells (arrows) (H&E, ×100). d Bizarre stromal cells with convoluted, large and hypochromatic nuclei, and apparent intranuclear pseudo inclusions are deceptively resembling dysplastic cells (H&E, ×500)
To the best of our knowledge, four cases of splenic hamartoma with bizarre stroma cells have been documented to date. Patients were found in the age range from 48 to 64, and showed female predilection (M:F = 1:3). Lesions’ greatest dimensions varied from 3.2 to 9 cm. The morphology and immunohistochemical profiles of the lesions were quite similar with our findings. All patients were successfully treated with splenectomy with benign clinical courses, as in our patient.
Conflict of interest Authors declare no conflict of interest.
13
Reference 1. Serra S, Chetty R. Bizarre stromal cells in ischemic bowel disease. Ann Diagn Pathol. 2005;9(4):193–6.
IMAGES IN HEMATOLOGY
Massive splenic hamartoma with bizarre stromal cells Nuri Yigit · Shannon Covey · Wayne Tam
Received: 28 November 2014 / Revised: 15 January 2015 / Accepted: 21 January 2015 © The Japanese Society of Hematology 2015
Keywords Splenic hamartoma · Bizarre stromal cells · Sickle cell trait
A 35-year-old female with history of sickle cell trait presented to the emergency room with chest pain and pelvic pressure. Blood test showed pancytopenia. She had already undergone a bone marrow biopsy, which was found with a norm cellular marrow with increased megakaryocytes. Computed tomography scan with contrast revealed massive splenomegaly, secondary to a heterogeneously hypo enhancing solitary lesion with multiple relatively hypo dense striations, measuring up to 24.5 × 13.6 × 13.0 cm. Differential included mainly hamartoma and lymphoma, with other benign and malignant entities considered less likely. The patient underwent splenectomy. Cut sections of spleen revealed a large well-circumscribed, dark red colored, soft and solid mass replacing nearly the entire spleen with a small rim of unremarkable parenchyma (Fig. 1a, b). Histopathological examination showed a
Present Address: N. Yigit (*) · S. Covey · W. Tam Department of Pathology and Laboratory Medicine, Weill Cornell Medical College/New York-Presbyterian Hospital, 525 East 68th Street Starr Pavilion, 715, New York, NY 10065, USA e-mail: [email protected]; [email protected] S. Covey e-mail: [email protected] W. Tam e-mail: [email protected] N. Yigit Department of Pathology, Gulhane Military Medical Academy and School of Medicine, Etlik Kecioren, Ankara 06010, Turkey
well-demarcated and encapsulated mass with focal infarcts, extensive hemorrhage, and extramedullary hematopoiesis, including erythropoiesis and megakaryopoiesis (Fig. 1c). Thick bands of hypocellular fibrosis, disorganized sinusoids, and vascular spaces separated by red-pulp stroma, without any white pulp elements, were observed. Many dispersed small lymphocytes, plasma cells, siderophage, and a few siderotic nodules (Gamna–Gandy bodies) were also noted in the background. The most striking population was scattered bizarre stromal cells, which were seen throughout the stroma without any association with vascular lumina. These cells composed of round to oval distorted or multilobulated nuclei, open chromatin, conspicuous nucleoli and scant cytoplasm (Fig. 1d). Large and pale intranuclear pseudo inclusions were frequently identified. Although their morphology was worrisome, they were devoid of some features of malignancy such as hyperchromasia, increased mitosis, or infiltrative growth pattern. An immunohistochemical panel containing CD34, CD31, CD8, CD163, CD138, Kappa, Lambda, CD30, S100, nerve growth factor receptor, smooth muscle actin, Desmin, and Pancytokeratin was performed. The bizarre cells were negative for all markers except focal and faint positivity for Desmin. Immunostains confirmed vascular and sinusoidal disorganization within the lesion. Plasma cells were polytypic for Kappa and Lambda light chains. Additionally, special stains for Grocott’s methenamine silver and acidfast bacilli were negative for any microorganism. Although these cells raised suspicion of malignancy, they were completely benign in nature. Their deceptively malignant morphology and lack of specific expression of any immunohistochemical marker are attributable to degenerative changes that can result from losing connections with microenvironmental elements and deteriorated nutritional supply [1].
13
N. Yigit et al.
Fig. 1 a, b Large lesion contains extensive hemorrhage and infarct foci. The clear demarcation line is seen between lesion and small intact rim of splenic parenchyma (arrows). c The hamartoma are harboring an admixture of extensive hemorrhages, hemosiderin-
laden macrophages, erythropoiesis foci (upper left), and bizarre cells (arrows) (H&E, ×100). d Bizarre stromal cells with convoluted, large and hypochromatic nuclei, and apparent intranuclear pseudo inclusions are deceptively resembling dysplastic cells (H&E, ×500)
To the best of our knowledge, four cases of splenic hamartoma with bizarre stroma cells have been documented to date. Patients were found in the age range from 48 to 64, and showed female predilection (M:F = 1:3). Lesions’ greatest dimensions varied from 3.2 to 9 cm. The morphology and immunohistochemical profiles of the lesions were quite similar with our findings. All patients were successfully treated with splenectomy with benign clinical courses, as in our patient.
Conflict of interest Authors declare no conflict of interest.
13
Reference 1. Serra S, Chetty R. Bizarre stromal cells in ischemic bowel disease. Ann Diagn Pathol. 2005;9(4):193–6.
