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Unusual biclonal IgA plasma cell myeloma with aberrant expression of high-risk immunophenotypes: first report of a new diagnostic and clinical challenge
Carlos A. Monroig-Rivera, Clara N. Finch Cruz
J Pathol Transl Med. 2023;57(2):132-137.   Published online March 14, 2023
DOI: https://doi.org/10.4132/jptm.2023.02.07
  • 1,929 View
  • 123 Download
AbstractAbstract PDF
IgA plasma cell myeloma (PCM) has been linked to molecular abnormalities that confer a higher risk for adverse patient outcomes. However, since IgA PCM only accounts for approximately 20% of all PCM, there are very few reports on high-risk IgA PCM. Moreover, no such reports are found on the more infrequent biclonal IgA PCM. Hence, we present a 65-year-old Puerto Rican female with acute abdominal pain, concomitant hypercalcemia, and acute renal failure. Protein electrophoresis with immunofixation found high IgA levels and detected a biclonal IgA gammopathy with kappa specificity. Histomorphologically, bone marrow showed numerous abnormal plasma cells (32%) replacing over 50% of the marrow stroma. Immunophenotyping analysis detected CD45-negative plasma cells aberrantly expressing CD33, CD43, OCT-2, and c-MYC. Chromosomal analysis revealed multiple abnormalities including the gain of chromosome 1q. Thus, we report on an unusual biclonal IgA PCM and the importance of timely diagnosing aggressive plasma cell neoplasms.
Abdominal Fibromatosis in a Young Child: A Case Study and Review of the Literature
Hyun Hee Chu, Pyoung Han Hwang, Yeon Jun Jeong, Myoung Ja Chung
Korean J Pathol. 2013;47(5):472-476.   Published online October 25, 2013
DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.5.472
  • 7,282 View
  • 38 Download
  • 3 Crossref
AbstractAbstract PDF

Fibromatoses comprise many different entities of well-differentiated fibroblastic proliferation with variable collagen production and form a firm nodular mass. Abdominal fibromatosis is distinguishable from other forms of fibromatosis because of its location and its tendency to occur in women of childbearing age during or following pregnancy. Abdominal fibromatosis in children is an extremely rare condition. A 15-month-old boy presented with an abdominal wall mass that had recently increased in size. Mass excision was perfomed. The tumor was 4.3×4.1 cm and partly circumscribed. Histologically, the tumor was composed of parallel long fascicles of spindle-cells with a uniform appearance. The edges of the resected mass were infiltrative, and the surgical margins were positive. Mitotic figures were <1/10 high power fields. No cellular atypia or necrosis was present. The tumor cells were positive for vimentin and nuclear β-catenin staining.

Citations

Citations to this article as recorded by  
  • A rare tumor of the large bowel in a young boy
    Shyam Srinivasan, Soumitra Saha
    Cancer Research, Statistics, and Treatment.2021; 4(4): 752.     CrossRef
  • Uncommon abdominal wall mass in a young boy: Desmoid tumor
    Levent Cankorkmaz, Mehmet H. Atalar, H. Reyhan Eğilmez
    Cumhuriyet Medical Journal.2018; : 811.     CrossRef
  • Lesiones ocupantes de espacio en pared abdominal (no herniaria). La visión del patólogo
    Isidro Machado, Julia Cruz, Javier Lavernia, Fernando Carbonell
    Revista Hispanoamericana de Hernia.2015; 3(3): 85.     CrossRef
Case Reports
Intra-abdominal Desmoplastic Small Round Cell Tumor Diagnosed by Lymph Node Biopsy: A case report.
Myung Jin Ju, Kwang Min Lee, Hye Kyung Lee, Dong Kyu Chung
Korean J Pathol. 1995;29(5):698-701.
  • 1,582 View
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AbstractAbstract
Intra-abdominal desmoplastic small round cell tumor has been described in the literature since 1989. It is characterized by the occurrence in ages less than 40 with male predominance, an intra-abdominal location, and small round to oval shaped tumor cells with divergent differentiation in the background of the desmoplastic stroma. We recently experienced this tumor in an inguinal lymph node of a 36-year-old man. It is suspected that it metastasized from a lower intra-abdominal tumor. Immunohistochemical stains for keratin, epithelial membrane antigen, vimentin, S-100 protein and neuron specific enolase were positive. This is the first documented case in Korea. Herein, we report on this tumor with a review of literature.
Intraabdominal Desmoplastic Small Cell Tumors with Divergent Differentiation: Report of two cases with immunohistochemical and ultrastructural studies.
Young Ha Oh, Nam Hoon Kim, Joo Seob Keum, Moon Hyang Park
Korean J Pathol. 1996;30(1):40-49.
  • 1,590 View
  • 20 Download
AbstractAbstract PDF
We studied two intraabdominal desmoplastic small cell tumors. The patients were two men, 37 and 23 years old, with jaundice and palpable abdominal masses. On exploratory laparotomy, each patient revealed a huge mass in the greater omentum with disseminated peritoneal seeding, measuring 32 cm and 11 cm in its greatest dimension, respectively. The tumor involved the diaphragm, rectal shelf, and cul de sac in case 1, and it involved the porta hepatis, retroperitoneum, and serosal surface of the ascending and transverse colon in case 2. Omentectomy of the huge mass and satellite masses was performed in each patient. Both tumors showed nearly the same histopathologic features. The histologic pattern was suggestive of a metastatic small cell carcinoma, but there was no specific, single primary site. The tumors consisted of variably sized, discrete islands of epithelial-like small cells in dense desmoplastic stroma. The tumor cells revealed divergent epithelial, mesenchymal, and neural differentiation by histologic, immunohistochemical, and electron microscopic observations. Only one cycle of chemotherapy including cisplatin and VP-16 was given in case 1 because of a subsequent hepatic problem, who, thereafter, showed massive intraabdominal recurrent tumors 6 months after diagnosis. In case 2, the poor condition of the patient had made chemotherapy and radiotherapy impossible. Case 2 died of disseminated intravascular coagulation following progressive cachexia 7 months after diagnosis.
Intraabdominal Heterotopic Thymus: Report of an autopsy case.
Hye Seung Han, Je Geun Chi
Korean J Pathol. 1996;30(11):1057-1059.
  • 1,634 View
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AbstractAbstract PDF
Ectopic thymus results from the aberrant migration of thymic tissue and is mostly present in the mediastinum, the base of the skull, the tracheal bifurcation and the cervical region. We report the first case of intraabdominal heterotopic thymus incidentally detected and attached to the liver without associated anomalies. This fetus was sent to the Department without any clinical information. The fetus was small for gestational age, but had no external abnormalities. Each organ showed normal development except for the liver. The liver weighed 6 gm(normal 17.064+/-4.143 gm). Gray white heterotopic thymus was attached to the superior surface of the liver in the subdiaphragmatic area. It measured 1.1x0.6x0.5 cm. There was no diaphragmatic defect. The cervical thymic tissue near the thyroid was small and measured 0.2 gm(normal 0.927+/-0.485 gm). There was no thymic tissue in the anterior superior mediastinum. The histologic features of the heterotopic thymus were identical to the orthotopic thymus showing features appropriate for the gestational age. The origin of this subdiaphragmatic heterotopic thymus is speculated.
Original Article
Intra-abdominal Angiosarcomatosis Induced by Irradiation.
Hee Jin Chang, Youn Joo Kim, Sung Eun Yang, Sung Sook Paeng, Jung Il Suh
Korean J Pathol. 1997;31(3):269-274.
  • 1,642 View
  • 14 Download
AbstractAbstract PDF
Therapeutic irradiation can induce angiosarcoma. Radiation-induced angiosarcoma constitutes 20% of all angiosarcomas. Although its common site of origin is the skin and subcutaneous tissue, it rarely arises in small or large bowels with a presentation as multifocal abdominal angiosarcomatosis. We report a case of intra-abdominal angiosarcomatosis involving the jejunum, ileum, transverse colon, mesentery and right ovary in a 63-year-old female. It developed 10 years after therapeutic irradiation for squamous cell carcinoma of uterine cervix. She developed panperitonitis due to intestinal perforation. She died from sepsis 3 days after segmental resection of the small bowel and right oophorectomy. We reviewed the previously reported cases and describe the clinicopathologic features of this tumor.
Case Reports
Extrapulmonary Silicosis: A case report.
Won Mi Lee, Se Jin Jang, Eun Kyung Hong, Jung Dal Lee
Korean J Pathol. 1998;32(7):540-542.
  • 1,920 View
  • 10 Download
AbstractAbstract
A case of extrapulmonary silicosis involving abdominal lymph nodes in a 62-year-old man is presented. The patient underwent subtotal gastrectomy and regional lymph nodes dissection for gastric adenocarcinoma of clinical stage III. On gross examination, two separate gastric adenocarcinomas and multiple enlarged lymph nodes mimicking metastatic lesion were noted. Microscopic examination of the enlaged lymph nodes revealed characteristic sclerohyaline silicotic nodules without metastatic adenocarcinoma. Chest roentgenogram showed diffusely scattered multiple tiny nodular lesions in the entire lung fields, particularly the posterior side of both upper lung fields. He had worked in a mine for 18 years and had been exposed to silica for 5 of those years. We report incidentally found, unusual case of extrapulmonary silicosis.
Fibromatosis of the Parotid Gland: A Case Report.
Dae Su Kim, Chulhwan Kim, Insun Kim
Korean J Pathol. 2002;36(2):112-114.
  • 1,923 View
  • 23 Download
AbstractAbstract PDF
A 51-year-old woman was presented with a palpable mass in the infraauricular area that had existed for 4-months. The mass from the superficial lobe of the parotid gland was 2.7 cm in the greatest dimension and was ill-defined with rubbery, homogeneous, and fibrotic appearance. Histologically, the lesion was infiltrative into the lobules of the paratid gland, and was composed of a proliferation of spindle or stellate cells with cellular and sclerotic areas. On immunohistochemistry, the cells were only positive for vimentin and focally for smooth muscle actin. Differential diagnosis from nerve sheath tumors and solitary fibrous tumors involving the parotid gland was emphasized.
Abdominal Endometriosis Diagnosed by Fine Needle Aspiration Cytology: A Report of Two Cases.
Myoung Ja Chung, Yeon Jun Jeong, Ho Myong Hwang, Kyu Yoon Jang, Woo Sung Moon, Myoung Jae Kang, Dong Geun Lee
Korean J Cytopathol. 2004;15(1):70-73.
  • 1,543 View
  • 16 Download
AbstractAbstract PDF
The incidence of endometriosis in post-operative abdominal scars is rare. We describe two cases of abdominal endometriosis diagnosed by fine needle aspiration (FNA). Both patients presented with subcutaneous masses at previous cesarean section scars with cyclic symptoms of pain. The cytologic smears were cellular and comprised two distinct cell populations consisting of epithelial and stromal components. An epithelial component consisted of flat sheets of polygonal cells and the second stromal component showed crowded clusters of spindle cells or isolated single cells. Hemosiderin-laden macrophages were found in the background. FNA offers a safe and effective tool for diagnosis of abdominal wall endometriosis.
Imprint Cytology of a Desmoplastic Small Round Cell Tumor: A Case Report.
Joon Hyuk Choi, Yong Jin Kim, Jae Hwang Kim
Korean J Cytopathol. 2007;18(1):81-86.
  • 1,784 View
  • 22 Download
AbstractAbstract PDF
Desmoplastic small round cell tumor (DSRCT) is a rare malignant mesenchymal neoplasm. It mainly involves the abdominal or pelvic peritoneum of male adolescents. We report here the imprint cytologic features of a case of DSRCT occurring in the intraabdominal cavity of a 21-year-old man. A microscopic examination showed moderate cellularity. The tumor cells were singly arranged and arranged in clusters. The cells had round to oval nuclei with finely granular chromatin, inconspicuous nucleoli and scanty cytoplasm. Some tumor cells showed nuclear molding, and some cells had an epitheloid appearance with a large amount of lightly eosinophilic cytoplasm. A rosette-like pattern was present. Spindle-shaped, fibroblastic stromal cells were occasionally found. The tumor cells were immunoreactive for the markers cytokeratin (AE1/AE3), epithelial membrane antigen (EMA), desmin, vimentin and neuron specific enolase (NSE).
Mesenteric Cystic Lymphangioma with Sustained Abdominal Pain : Report of a case.
Gyeong Hoon Kang, Yong Il Kim, Woo Ho Kim, In Sung Song, Kyoo Wan Choi
Korean J Pathol. 1991;25(5):488-490.
  • 1,606 View
  • 11 Download
AbstractAbstract
A case of cystic lymphangioma of the mesentery with severe and persistent abdominal pain in a 22-year-old man is presented. The cyst was filled with chylous fluid. Microscopically, numerous nerve bundles were incorporated within the lymphangiomatous walls, and some protruded into the lumen. The above findings lead to a suggestion that mesenteric lymphangioma may differ from those in the other sites by its abundance of incorporation of nerve bundles into the lymphangiomatous walls, and that increase of tumor size by intracystic accumulation of chylous fluid may subsequently result in increase of intraluminal pressure to compress the nerve bundles with which abdominal pain is much enhanced.

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