Harinder Sawhney

A bilateral primary yolk sac tumor of the lung associated with chromosome 3 polysomy: understanding the Oct 3/4 and Sox 2 interaction.

Extragonadal germ cell tumors (EGCTs) in the lung are extremely rare and their pathogenesis is poorly understood. We report a case in a 48-year-old female which was very aggressive and stained positive for primoridial germ cell markers. Interestingly, there was chromosome 3 polysomy noted. To our knowledge this is the first chromosomal aberration noted in a primary germ cell tumor of the lung.

Spontaneous Acute Tumour Lysis Syndrome in Gastric Adenocarcinoma: A Case Report and Literature Review

Acute tumour lysis syndrome (ATLS) is one of the lifethreatening complications of chemotherapy for cancers. It has typical biochemical finding of hyperuricaemia, acute renal failure, hyperkalaemia, hypophosphataemia and hypocalcaemia. These findings are caused by disintegration of tumour cells and subsequent release of their content in to the blood [1]. ATLS is usually seen in tumours with heavy cell turnover and large burden. It is because of this reason, ATLS is primarily seen in cancers of the blood but it has also been more increasingly recognized in solid tumours. Recently, ATLS has been described in relation to cancers of the colon [2], gall bladder [3], biliary tract [4], skin [5] and breast [6]. Here, we describe an exceedingly rare case of spontaneous ATLS in a patient with gastric adenocarcinoma. To our knowledge, only one case of spontaneous ATLS has been described previously in literature with adenocarcinoma of the stomach which was reported more than a decade ago [7].

Extramedullary hemopoiesis with littoral cell angioma involving main and accessory spleens

Dear Editor, Extramedullary hemopoiesis is not a known entity associated with littoral cell angioma (LCA). We report a case of extramedullary hemopoiesis in LCA involving main and accessory spleens. A 44-year-old lady with a history of type 2 diabetes mellitus was admitted to hospital for vague abdominal pain. She denied fever, chills, or night sweats and there was no lymphadenopathy. Physical examination was unremarkable. All hematological and biochemical tests, including LDH, were normal. Enhanced CT scan showed multiple lesions in the spleen. MRI of the abdomen after the administration of intravenous gadolinium was highly suggestive of benign splenic lesions. Gallium scintigraphy revealed no abnormal uptake in either the spleen or other organs. Subsequently, a CT-guided biopsy and pathological examination revealed LCA. Immunohistochemical stains were positive for CD31, CD68, and CD21, while CD34 was negative, confirming this diagnosis. Splenectomy was done; the spleen measured 11×9×3 cm and it showed multiple nodules of LCA, the largest measures 3.5×2×2 cm with focal extramedullary hemopoiesis with megakaryocytes (Fig. 1). Two accessory spleens were found and one of them was involved by LCA. The other reasons for extramedullary hemopoiesis were excluded. Themost common primary tumors of the spleen are vascular in origin. These can be divided to three groups depending on the level of aggressiveness. The benign tumors include hemangioma, hamartoma, and lymphangioma; tumors of variable or uncertain biologic behavior include LCA, hemangioendothelioma, and hemangiopericytoma; whereas malignant tumors include angiosarcoma [1]. A malignant subtype of LCA, littoral cell angiosarcoma, has also been reported [2]. LCAs may occur at any age without gender predilection. Clinically, patients with LCA present with an abdominal mass from splenomegaly, symptoms of hypersplenism such as anemia and thrombocytopenia, or constitutional symptoms like low-grade fever and fatigue. Radiographically and at gross pathology, an enlarged spleen containing multiple nodules is most commonly seen. On unenhanced CT scan, the lesion is hypodense, but can be imperceptible. Only on delayed images does the entire lesion enhance, suggesting a vascular lesion [3]. The diagnosis of LCA is confirmed histologically and by immuno-histochemistry. LCA is characterized histologically by anastomosing vascular channels with pseudopapillary aspect and dilated cavernous dilated space. Plump exfoliated cells are often found within the lumina of vascular channels. Foci of hemosiderin pigment, calcification, and extramedullary erythropoiesis have been reported [4]. Immunophenotypically, LCA are positive for endothelical markers CD31, CD21, factor VIII-related antigen, and ulex europeaus and histiocyte marker CD68. S-100 positivity has also been studied in a few cases of LCA [5]. LCA has also been described in association with severe aplastic anemia, myelodysplastic syndrome, renal transplant, liver cirrhosis, autoimmune conditions, metabolic disorders, chronic infections like hepatitis B and C, and inherited conditions like Gaucher disease and Epstein’s syndrome [6–8]. Cases of LCA that develop after chemotherapy and immune suppressive therapy have also been reported. Most LCAs are seen in main spleen and only one case of LCA involving accessory spleen has been reported [9]. Extramedullary hemopoiesis has been reported in renal cell carcinoma, perirenal liposarcoma, spindle cell lipoma of neck, idiopathic myelofibrosis, hepatic angiomyolipoma, and in Ann Hematol (2007) 86:695–696 DOI 10.1007/s00277-007-0311-9

Clinical progression of multiple myeloma presenting as parotid gland plasmacytoma

Plasmacytomas are tumors of plasma cells which can be divided into medullary or extramedullary type depending on the site where they arise. Parotid gland plasmacytoma is rarely described entity as initial presentation of multiple myeloma [1, 2]. Here we describe a patient who had clinical progression of multiple myeloma as parotid gland plasmacytoma. To our knowledge this is the first case described in literature where parotid gland plasmacytoma presented as clinical progression of multiple myeloma since parotid plasmacytoma was discovered before the diagnosis of multiple myeloma in all previous cases [3, 4]. Our patient is a 62-year-old female who was diagnosed with IgG kappa multiple myeloma 8 years ago and was previously treated with multiple chemotherapies including thalidomide, bortezomib and VAD (vincristine–adriamycin–dexamethasone) regimen, came to hematology clinic with slowly growing, painless mass in front of her right ear from past 3 months. On examination, this swelling was firm in consistency with smooth margins, non-tender, about 2 9 2 cm in size and appeared to be originating from parotid gland. No adenopathy was noted and examination of head and neck was otherwise unremarkable. A CT scan of soft tissue of neck was performed which showed a solid mass in right parotid gland which measured 2.5 9 2.0 cm with smooth margins and normal left parotid gland. Subsequently, a right superficial parotidectomy with conservation of facial nerve was performed. Histopathologic examination revealed glandular tissue with attached fibro-connective tissue with sheets of innumerous plasma cells consistent with extramedullary plasmacytoma (Fig. 1). Immunohistochemical studies for CD138, IgG and Kappa were strongly positive and were negative for IgM, IgA and Lambda consistent with previously diagnosed IgG kappa multiple myeloma (Fig. 2). Patient’s blood tests including immunoelectrophoresis and quantitative immunoglobulins did not show any significant differences when compared to the blood tests which were conducted 7 months ago. A total body skeletal survey was performed which showed generalized osteopenia, endosteal scalloping and wedge deformities in multiple vertebrae, but there was no evidence of bony lytic lesion or any other bony progression. This patient did not undergo chemotherapy or radiotherapy after parotidectomy as there was neither biochemical nor bony progression of multiple myeloma. Multiple myeloma can be present as plasmacytoma at the time of diagnosis [4] or at the time of subsequent relapse during the course of disease. Disease recurrence is commonly seen in medullary sites, but extramedullary sites are increasingly becoming common. To our knowledge this is the first case described in literature where parotid gland plasmacytoma presented as clinical progression of multiple myeloma. This case also reiterates the importance of physical exam in patients with hematological malignancies.

A vanishing act: the incredible erlotinib.

Sir, A 57-year-old male with 100-pack-year smoking history came to our hospital with shortness of breath. Imaging revealed multiple bilateral nodules (Panel a) which on biopsy showed a focally mucin producing, broncho-alveoloar carcinoma [Figure 1]. He was started on cisplatinum and gemcitabine. After 2 cycles, there was no evident reduction in the bulk of the disease. Mutational analysis studies suggested EGFR mutation (deletion of exon 19) and the patient was then started on erlotinib monotherapy. The follow up chest X-ray done at 3 months post erlotinib therapy is shown in Panel b [Figure 1].