Letters to Editor Departments of Orthopedic, and 1Pathology, Pt BD Sharma PGIMS, Rohtak, Haryana, India
Correspondence to:
Dr. Zile Singh Kundu, E‑mail: [email protected]
References 1. 2. 3. 4. Figure 7: The widely excised specimen
We conclude that ASPS can remain undetected for a long period and can be easily overlooked due to relative lack of symptoms of the primary tumor. Early metastasis is a characteristic feature of this poor prognostic tumor and unfortunately, in large number of patients, it present with lung or brain metastasis as the first manifestation of the disease. In the cases of occult primary with metastasis to the lungs, ASPS should also be suspected as one of the possibility.
Fletcher CD. Unni KK, Mertens F. Alveolar soft part sarcoma. Pathology and Genetics of Tumors of Soft tissue and Bone. Lyon: IARC Press; 2002. p. 208‑10. Weiss SW, Goldblum Jr. Malignant soft tissue tumors of uncertain type. In: Enzinger and Weiss’s Soft Tissue Tumor. 4th ed. St Louis: CV Mosby; 2001. p. 1483‑571. Montgomery E. Soft tissue tumor. In: Silverberg Principles and Practice of Surgical Pathology and Cytopathology. 4th ed. Philadelphia: Churchill Livingstone Elsevier; 2006. p. 307‑418. Logrono R, Wojtowycz MM, Wunderlich DW, Warner TF, Kurtycz DF. Fine needle aspiration cytology and core biopsy in the diagnosis of alveolar soft part sarcoma presenting with lung metastases. A case report. Acta Cytol 1999;43:464‑70.
Access this article online Quick Response Code:
Website: www.indianjcancer.com DOI: 10.4103/0019-509X.146775 PMID: *****
Kundu ZS, Kalra R1, Rana P1, Mathur SK1, Sangwan SS, Siwach RC
Long term survival after whole brain radiotherapy for brain metastasis in follicular dendritic cell sarcoma
machine. He improved symptomatically and a subsequent CECT scan showed near complete regression of the space occupying lesions as well edema. Patient has been on a regular follow‑up since then and is largely asymptomatic. His most recent CECT scan of the head (May 2013) is within the normal limits [Figure 2]. The patient is therefore clinically and radiologically free of disease, 7 years after the initial diagnosis and 6 years following radiotherapy for brain metastasis.
A 36‑year‑old male patient presented with epigastric pain, anorexia and weight loss of 1 month duration (July 2006). Physical examination revealed epigastric fullness and a right sided inguinal lymph node measuring 6‑cm in greatest dimension. Fine‑needle aspiration from the inguinal lymph node and a biopsy revealed FDCS. Contrast enhanced computed tomographic scan (CECT scan) of the abdomen revealed multiple enlarged retroperitoneal, mesenteric and right inguinal lymph nodes. Further metastatic work‑up was non‑contributory. Patient was thus started on chemotherapy for disseminated dendritic cell sarcoma of the lymph nodes. He received eight cycles of adriamycin and ifosfamide combination chemotherapy. A subsequent CECT scan showed complete resolution of lymph node masses. The patient was asymptomatic for 1‑year when he presented with headache and vomiting. CECT scan of the head was highly suggestive of metastasis in the frontal region [Figure 1]. After a course of steroids, mannitol and neurosurgery consultation for decompression, the patient was taken up for palliative WBRT. He received 30 Gy in 10 daily fractions by parallel opposed portals on a telecobalt
Discussion
Sir, We present a rare case of brain metastasis in a patient with follicular dendritic cell sarcoma (FDCS), who was treated with whole brain radiotherapy (WBRT) and is alive and free from disease, 7 years after diagnosis.
Indian Journal of Cancer | July–September 2014 | Volume 51 | Issue 3
Dendritic cell sarcoma is a rare neoplasm of unknown etiology first reported in 1978. [1] Since then 462 cases have been reported excluding the present one.[2] Systemic involvement and distant metastasis are more commonly seen in liver and lungs. Brain involvement is extremely rare and has been reported in four patients only (including the present one). FDCS is the most common histological subtype (343 reported cases). It behaves like an intermediate grade sarcoma with a substantial risk of local recurrence (28%) and distant metastasis (27%). [2] For localized disease, a radical excision is the preferred treatment and there is no further benefit with adjuvant radiotherapy and chemotherapy. For disseminated/advanced disease (like the present case), chemotherapy is the only option, but the outcome is generally poor.[2] Brain metastases develop in 20‑40% cancer patients during their course of illness. [3] The prognosis for such patients is generally poor with a median survival of less than 3 months without any treatment. The mainstay of treatment has been corticosteroids for alleviation of 395
Letters to Editor
Figure 1: Ill‑defined hypo‑dense space occupying lesion with necrotic center in the mid frontal region and associated bilateral frontal lobe edema causing partial obliteration of bilateral frontal horns and effacement of frontal lobe sulci and gyri
peritumoral edema and WBRT for growth restraint, symptomatic relief and possibly improvement in survival.[3] The overall response rate to WBRT ranges from 64% to 85% and the median survival approximately 6 months.[4] Moreover, a recently published Cochrane review on WBRT did not show survival benefit with the usage of different fractionation schedules as against the “standard” 30 Gy in 10 daily fractions, also used in the present case, radio‑sensitizers and radiosurgery boost.[4] Contrary to the outcomes reported in the literature, there was a complete radiological response in this case and the patient and is alive and free from disease 6 years after WBRT.
Figure 2: A normal contrast enhanced computed tomographic brain of the same patient, conducted 6‑years after whole brain radiotherapy for brain metastasis in dendritic cell carcinoma
References 1. Lennert K, Mohri N. Histopathology and diagnosis of non Hodgkin’s lymphomas. In: Vehrlinger E, editor. Malignant Lymphomas Other than Hodgkin’s Disease. Berlin, Germany: Springer‑Verlag; 1978. p. 448. 2. Saygin C, Uzunaslan D, Ozguroglu M, Senocak M, Tuzuner N. Dendritic cell sarcoma: A pooled analysis including 462 cases with presentation of our case series. Crit Rev Oncol Hematol 2013;13:100‑5. 3. Wong J, Hird A, Kirou‑Mauro A, Napolskikh J, Chow E. Quality of life in brain metastases radiation trials: A literature review. Curr Oncol 2008;15:25‑45. 4. Tsao MN, Lloyd N, Wong RK, Chow E, Rakovitch E, Laperriere N, et al. Whole brain radiotherapy for the treatment of newly diagnosed multiple brain metastases. Cochrane Database Syst Rev 2012;4:1‑73. Access this article online Quick Response Code:
Dimri K, Trehan R, Pandey AK, Khosla D
DOI: 10.4103/0019-509X.146778
Department of Radiotherapy, Government Medical College, Chandigarh, India
PMID: *****
Correspondence to:
Dr. Kislay Dimri, E‑mail: [email protected]
Psuedo Chediak Higashi anomaly in case of hypogranular variant of acute promyelocytic leukemia (AML‑M3v): A morphologic enigma
Sir, A 50-year-old lady, labourer by occupation, presented to our hospital with fever, pain in abdomen and increased frequency of micturition of 15 days duration, along with recent onset of breathlessness and chest pain for the past three days. She also gave a history of being treated outside for urinary tract infection (UTI) with norfloxacin. On examination, she was febrile (100°F) with a pulse of 100/min, blood pressure of 110/80 mm of Hg, and respiratory rate of 28/min. Patient was pale, poorly hydrated and had oral candidiasis. Respiratory examination revealed bilateral basal crepitations with spO2 of 82% at room air. Rest of the examination was within normal limits and the patient was admitted to High Dependency Unit with a provisional diagnosis of sepsis with Acute Respiratory 396
Website: www.indianjcancer.com
Distress Syndrome (ARDS). Investigations revealed hypokalemia (serum K+ level of 1.9 mEq/l) and respiratory alkalosis (pH ‑ 7.6 pH units, pO2 ‑ 49.1 mm Hg, pCO2 - 29 mm Hg and HCO3 - 30.3 mEq/l). Chest X‑ ray showed changes of early ARDS and urine culture grew E. Coli which was sensitive to amikacin and doxycycline but resistant to norfloxacin and ampicillin. Hematologic examination at this stage revealed hemoglobin 6.7 gm%, total leukocyte count 52,700/cumm and platelet count 1,13,000/cumm. Differential count revealed 54 atypical promyelocyte count, 07 myelocyte, 30 neutrophils and 09 lymphocytes. These atypical promyelocytes were large with bilobed nuclei and three to four nucleoli. These cells had moderate amount of basophilic cytoplasm which was hypogranular with occasional Auer rods. About 2% of these atypical cells showed round to oval, azurophilic intracytoplasmic granules with size ranging from 1‑3 µm [Figures 1and 2]. These granules were not seen in any other cell. The atypical promyelocytes showed strong Myeloperoxidase (MPO) positivity as did these giant Indian Journal of Cancer | July–September 2014 | Volume 51 | Issue 3
Copyright of Indian Journal of Cancer is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Correspondence to:
Dr. Zile Singh Kundu, E‑mail: [email protected]
References 1. 2. 3. 4. Figure 7: The widely excised specimen
We conclude that ASPS can remain undetected for a long period and can be easily overlooked due to relative lack of symptoms of the primary tumor. Early metastasis is a characteristic feature of this poor prognostic tumor and unfortunately, in large number of patients, it present with lung or brain metastasis as the first manifestation of the disease. In the cases of occult primary with metastasis to the lungs, ASPS should also be suspected as one of the possibility.
Fletcher CD. Unni KK, Mertens F. Alveolar soft part sarcoma. Pathology and Genetics of Tumors of Soft tissue and Bone. Lyon: IARC Press; 2002. p. 208‑10. Weiss SW, Goldblum Jr. Malignant soft tissue tumors of uncertain type. In: Enzinger and Weiss’s Soft Tissue Tumor. 4th ed. St Louis: CV Mosby; 2001. p. 1483‑571. Montgomery E. Soft tissue tumor. In: Silverberg Principles and Practice of Surgical Pathology and Cytopathology. 4th ed. Philadelphia: Churchill Livingstone Elsevier; 2006. p. 307‑418. Logrono R, Wojtowycz MM, Wunderlich DW, Warner TF, Kurtycz DF. Fine needle aspiration cytology and core biopsy in the diagnosis of alveolar soft part sarcoma presenting with lung metastases. A case report. Acta Cytol 1999;43:464‑70.
Access this article online Quick Response Code:
Website: www.indianjcancer.com DOI: 10.4103/0019-509X.146775 PMID: *****
Kundu ZS, Kalra R1, Rana P1, Mathur SK1, Sangwan SS, Siwach RC
Long term survival after whole brain radiotherapy for brain metastasis in follicular dendritic cell sarcoma
machine. He improved symptomatically and a subsequent CECT scan showed near complete regression of the space occupying lesions as well edema. Patient has been on a regular follow‑up since then and is largely asymptomatic. His most recent CECT scan of the head (May 2013) is within the normal limits [Figure 2]. The patient is therefore clinically and radiologically free of disease, 7 years after the initial diagnosis and 6 years following radiotherapy for brain metastasis.
A 36‑year‑old male patient presented with epigastric pain, anorexia and weight loss of 1 month duration (July 2006). Physical examination revealed epigastric fullness and a right sided inguinal lymph node measuring 6‑cm in greatest dimension. Fine‑needle aspiration from the inguinal lymph node and a biopsy revealed FDCS. Contrast enhanced computed tomographic scan (CECT scan) of the abdomen revealed multiple enlarged retroperitoneal, mesenteric and right inguinal lymph nodes. Further metastatic work‑up was non‑contributory. Patient was thus started on chemotherapy for disseminated dendritic cell sarcoma of the lymph nodes. He received eight cycles of adriamycin and ifosfamide combination chemotherapy. A subsequent CECT scan showed complete resolution of lymph node masses. The patient was asymptomatic for 1‑year when he presented with headache and vomiting. CECT scan of the head was highly suggestive of metastasis in the frontal region [Figure 1]. After a course of steroids, mannitol and neurosurgery consultation for decompression, the patient was taken up for palliative WBRT. He received 30 Gy in 10 daily fractions by parallel opposed portals on a telecobalt
Discussion
Sir, We present a rare case of brain metastasis in a patient with follicular dendritic cell sarcoma (FDCS), who was treated with whole brain radiotherapy (WBRT) and is alive and free from disease, 7 years after diagnosis.
Indian Journal of Cancer | July–September 2014 | Volume 51 | Issue 3
Dendritic cell sarcoma is a rare neoplasm of unknown etiology first reported in 1978. [1] Since then 462 cases have been reported excluding the present one.[2] Systemic involvement and distant metastasis are more commonly seen in liver and lungs. Brain involvement is extremely rare and has been reported in four patients only (including the present one). FDCS is the most common histological subtype (343 reported cases). It behaves like an intermediate grade sarcoma with a substantial risk of local recurrence (28%) and distant metastasis (27%). [2] For localized disease, a radical excision is the preferred treatment and there is no further benefit with adjuvant radiotherapy and chemotherapy. For disseminated/advanced disease (like the present case), chemotherapy is the only option, but the outcome is generally poor.[2] Brain metastases develop in 20‑40% cancer patients during their course of illness. [3] The prognosis for such patients is generally poor with a median survival of less than 3 months without any treatment. The mainstay of treatment has been corticosteroids for alleviation of 395
Letters to Editor
Figure 1: Ill‑defined hypo‑dense space occupying lesion with necrotic center in the mid frontal region and associated bilateral frontal lobe edema causing partial obliteration of bilateral frontal horns and effacement of frontal lobe sulci and gyri
peritumoral edema and WBRT for growth restraint, symptomatic relief and possibly improvement in survival.[3] The overall response rate to WBRT ranges from 64% to 85% and the median survival approximately 6 months.[4] Moreover, a recently published Cochrane review on WBRT did not show survival benefit with the usage of different fractionation schedules as against the “standard” 30 Gy in 10 daily fractions, also used in the present case, radio‑sensitizers and radiosurgery boost.[4] Contrary to the outcomes reported in the literature, there was a complete radiological response in this case and the patient and is alive and free from disease 6 years after WBRT.
Figure 2: A normal contrast enhanced computed tomographic brain of the same patient, conducted 6‑years after whole brain radiotherapy for brain metastasis in dendritic cell carcinoma
References 1. Lennert K, Mohri N. Histopathology and diagnosis of non Hodgkin’s lymphomas. In: Vehrlinger E, editor. Malignant Lymphomas Other than Hodgkin’s Disease. Berlin, Germany: Springer‑Verlag; 1978. p. 448. 2. Saygin C, Uzunaslan D, Ozguroglu M, Senocak M, Tuzuner N. Dendritic cell sarcoma: A pooled analysis including 462 cases with presentation of our case series. Crit Rev Oncol Hematol 2013;13:100‑5. 3. Wong J, Hird A, Kirou‑Mauro A, Napolskikh J, Chow E. Quality of life in brain metastases radiation trials: A literature review. Curr Oncol 2008;15:25‑45. 4. Tsao MN, Lloyd N, Wong RK, Chow E, Rakovitch E, Laperriere N, et al. Whole brain radiotherapy for the treatment of newly diagnosed multiple brain metastases. Cochrane Database Syst Rev 2012;4:1‑73. Access this article online Quick Response Code:
Dimri K, Trehan R, Pandey AK, Khosla D
DOI: 10.4103/0019-509X.146778
Department of Radiotherapy, Government Medical College, Chandigarh, India
PMID: *****
Correspondence to:
Dr. Kislay Dimri, E‑mail: [email protected]
Psuedo Chediak Higashi anomaly in case of hypogranular variant of acute promyelocytic leukemia (AML‑M3v): A morphologic enigma
Sir, A 50-year-old lady, labourer by occupation, presented to our hospital with fever, pain in abdomen and increased frequency of micturition of 15 days duration, along with recent onset of breathlessness and chest pain for the past three days. She also gave a history of being treated outside for urinary tract infection (UTI) with norfloxacin. On examination, she was febrile (100°F) with a pulse of 100/min, blood pressure of 110/80 mm of Hg, and respiratory rate of 28/min. Patient was pale, poorly hydrated and had oral candidiasis. Respiratory examination revealed bilateral basal crepitations with spO2 of 82% at room air. Rest of the examination was within normal limits and the patient was admitted to High Dependency Unit with a provisional diagnosis of sepsis with Acute Respiratory 396
Website: www.indianjcancer.com
Distress Syndrome (ARDS). Investigations revealed hypokalemia (serum K+ level of 1.9 mEq/l) and respiratory alkalosis (pH ‑ 7.6 pH units, pO2 ‑ 49.1 mm Hg, pCO2 - 29 mm Hg and HCO3 - 30.3 mEq/l). Chest X‑ ray showed changes of early ARDS and urine culture grew E. Coli which was sensitive to amikacin and doxycycline but resistant to norfloxacin and ampicillin. Hematologic examination at this stage revealed hemoglobin 6.7 gm%, total leukocyte count 52,700/cumm and platelet count 1,13,000/cumm. Differential count revealed 54 atypical promyelocyte count, 07 myelocyte, 30 neutrophils and 09 lymphocytes. These atypical promyelocytes were large with bilobed nuclei and three to four nucleoli. These cells had moderate amount of basophilic cytoplasm which was hypogranular with occasional Auer rods. About 2% of these atypical cells showed round to oval, azurophilic intracytoplasmic granules with size ranging from 1‑3 µm [Figures 1and 2]. These granules were not seen in any other cell. The atypical promyelocytes showed strong Myeloperoxidase (MPO) positivity as did these giant Indian Journal of Cancer | July–September 2014 | Volume 51 | Issue 3
Copyright of Indian Journal of Cancer is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
